AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesInformation ScienceHealth Care
Primary MyelofibrosisJanus Kinase 2Thrombocythemia, EssentialPolycythemia VeraMyeloproliferative DisordersReceptors, ThrombopoietinSplenomegalyMyelodysplastic-Myeloproliferative DiseasesThrombocytosisSea-Blue Histiocyte SyndromeBone MarrowMegakaryocytesMutationPrognosisKaryotypingHematopoiesis, ExtramedullaryPlatelet CountWorld Health OrganizationMutation, MissenseReticulinAmino Acid SubstitutionOsteosclerosisDisease ProgressionSurvival RateMultiple Sclerosis, Chronic Progressive
Primary Myelofibrosis
A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.
Janus Kinase 2
A Janus kinase subtype that is involved in signaling from GROWTH HORMONE RECEPTORS; PROLACTIN RECEPTORS; and a variety of CYTOKINE RECEPTORS such as ERYTHROPOIETIN RECEPTORS and INTERLEUKIN RECEPTORS. Dysregulation of Janus kinase 2 due to GENETIC TRANSLOCATIONS have been associated with a variety of MYELOPROLIFERATIVE DISORDERS.
Thrombocythemia, Essential
A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
Polycythemia Vera
A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.
Myeloproliferative Disorders
Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.
Receptors, Thrombopoietin
Cell surface receptors that are specific for THROMBOPOIETIN. They signal through interaction with JANUS KINASES such as JANUS KINASE 2.
Splenomegaly
Enlargement of the spleen.
Myelodysplastic-Myeloproliferative Diseases
Clonal myeloid disorders that possess both dysplastic and proliferative features but are not properly classified as either MYELODYSPLASTIC SYNDROMES or MYELOPROLIFERATIVE DISORDERS.
Thrombocytosis
Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)
Sea-Blue Histiocyte Syndrome
A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.
Bone Marrow
The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.
Megakaryocytes
Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.
Mutation
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Prognosis
A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.
Karyotyping
Mapping of the KARYOTYPE of a cell.
Hematopoiesis, Extramedullary
The formation and development of blood cells outside the BONE MARROW, as in the SPLEEN; LIVER; or LYMPH NODES.
Platelet Count
The number of PLATELETS per unit volume in a sample of venous BLOOD.
World Health Organization
A specialized agency of the United Nations designed as a coordinating authority on international health work; its aim is to promote the attainment of the highest possible level of health by all peoples.
Mutation, Missense
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
Reticulin
A scleroprotein fibril consisting mostly of type III collagen. Reticulin fibrils are extremely thin, with a diameter of between 0.5 and 2 um. They are involved in maintaining the structural integrity in a variety of organs.
Amino Acid Substitution
The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.
Osteosclerosis
An abnormal hardening or increased density of bone tissue.
Disease Progression
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
Survival Rate
The proportion of survivors in a group, e.g., of patients, studied and followed over a period, or the proportion of persons in a specified group alive at the beginning of a time interval who survive to the end of the interval. It is often studied using life table methods.
Multiple Sclerosis, Chronic Progressive
A form of multiple sclerosis characterized by a progressive deterioration in neurologic function which is in contrast to the more typical relapsing remitting form. If the clinical course is free of distinct remissions, it is referred to as primary progressive multiple sclerosis. When the progressive decline is punctuated by acute exacerbations, it is referred to as progressive relapsing multiple sclerosis. The term secondary progressive multiple sclerosis is used when relapsing remitting multiple sclerosis evolves into the chronic progressive form. (From Ann Neurol 1994;36 Suppl:S73-S79; Adams et al., Principles of Neurology, 6th ed, pp903-914)

Allogeneic stem cell transplantation for agnogenic myeloid metaplasia: a European Group for Blood and Marrow Transplantation, Societe Francaise de Greffe de Moelle, Gruppo Italiano per il Trapianto del Midollo Osseo, and Fred Hutchinson Cancer Research Center Collaborative Study. (1/532)

Agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder in which patients with poor prognostic features, receiving conventional treatments, have a median survival of less than 3 years. In this retrospective multicenter study, we analyze the results and try to define the indications for allogeneic stem cell transplantation in AMM. From January 1979 to November 1997, 55 patients with a median age of 42 years were transplanted from HLA-matched related (n = 49) or alternative (n = 6) donors for AMM. A multivariate analysis was conducted to identify factors associated with posttransplant outcome. The median posttransplant follow-up was 36 months (range, 6 to 223). The 5-year probability of survival was 47% +/- 8% for the overall group, and 54% +/- 8% for patients receiving an unmanipulated HLA-matched related transplant. The 1-year probability of transplant-related mortality was 27% +/- 6%. Hemoglobin level +info)

Jugular vein thrombosis: a rare presentation of atypical chronic myeloproliferative disorder in a young woman. (2/532)

Venous thromboembolism is common in subjects with chronic myeloproliferative disorders and is a recognized presenting feature of occult myeloproliferation. We report the case of a young woman who presented with acute thrombosis in the right jugular vein and pulmonary embolism. Splenomegaly and myeloid proliferation with bone marrow fibrosis, in the absence of the criteria for typical myeloproliferative disorders, allowed a diagnosis of an atypical form of chronic myeloproliferative disorder. This form carries a high risk of thrombosis and venous thromboembolism can be the presenting feature, though the course is often indolent. Acute thrombosis in the right jugular vein has not been so far described in these subjects. The outcome of young people with myelofibrosis is unpredictable, but a normal level of hemoglobin and the absence of blast cells and constitutional symptoms at presentation identifies subjects with a low probability of rapid disease progression.  (+info)

Dibromomannitol in the treatment of chronic granulocytic leukemia: a prospective randomized comparison with busulfan. (3/532)

Dibromomannitol (DBM) is a new agent for the treatment of chronic granulocytic leukemia. A propsective evaluation of the drug was undertaken in a randomized comparison with busulfan. Forty previously untreated, Philadelphia chromosome-positive cases were treated, with 20 patients in each treatment group. The protocol provided for continuous maintenance therapy after remission induction, with a crossover to the opposite drug in patients who became refractory to the primary agent but are without evidence of blastic tranformation. There were 14 remissions in the DBM group and 15 in those treated with busulfan. The rate of decrease of the elevated leukocyte count was more rapid with DBM, but prolonged disease control off treatment occurred in only three of 14 cases as opposed to nine of fifteen busulfan-treated patients who required a median delay of 12 mo before maintenance could be initiated. Hypoplasia occurred in one DBM patient and two busulfan cases. Following recovery, crossover to the opposite drug in two cases again resulted in hypopllasia. Increased skin pigmentation, amenorrhea, pulmonary fibrosis, and cytologic dysplasia, commonly associated with busulfan adminstration, were also noted with DBM. The median duration of disease control with busulfan was 34 mo and 26 mo with DBM. There was no signigicant difference in the incidence of blastic transformation, and median survival for both groups was 44 mo. DBM appears to be as effective as busulfan in the treatment of the chronic phase of CGL but with a more predictable myelosuppressive action. The principal advantage of busulfan over DBM is the fact that more than half the busulfan-treated patients experienced prolonged disease control off treatment.  (+info)

Myelofibrosis with myeloid metaplasia: diagnostic definition and prognostic classification for clinical studies and treatment guidelines. (4/532)

PURPOSE: Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and extramedullary hematopoiesis. Recent studies provide definite diagnostic criteria and prognostic classifications of the disease, and allogeneic stem-cell transplantation (SCT) now offers a chance of curing the disease. In order to put diagnostic criteria and prognostic classifications of the disease into the perspective of developing guidelines for treatment strategies, all studies published in the English literature over the last 30 years were reviewed. MATERIALS AND METHODS: Studies were identified through a MEDLINE search (1966 to present) and from the bibliographies of relevant articles. RESULTS: The Italian Consensus Conference on diagnostic criteria is a structured enterprise aimed at formulating a definition of MMM that will be used for enrolling patients onto clinical studies. It relies on the obligatory presence of myelofibrosis and on the exclusion of the BCR-ABL rearrangement or Philadelphia chromosome, in association with combinations of traditional features. Prognostic scores allow us to identify classes of patients on the basis of hemoglobin, age, WBC count, and chromosomal abnormalities. Several nonrandomized studies have indicated that allogeneic SCT for patients under the age of 55 is effective in prolonging survival in more than 50% of cases and in possibly curing the disease. Patients with the most severe prognosis are candidates. CONCLUSION: "Consensus" methodology offers a definition of MMM useful for conducting and reporting clinical studies. A detailed knowledge of prognostic factors can help to delineate guidelines for addressing patients with allogeneic SCT.  (+info)

Splenic myeloid metaplasia, histiocytosis, and hypersplenism in the dog (65 cases). (5/532)

Splenectomy specimens from 65 dogs with severe, diffuse, sustained, and progressive splenomegaly were examined. The clinical signs, hematology, and serum chemistry values in for the dogs were not useful diagnostic features. Microscopic changes in the spleens were distinctive and consisted of 1) myeloid metaplasia, 2) histiocytosis, 3) erythrophagocytosis, and 4) thrombosis with segmental infarction. Ultrastructural features suggested proliferative changes in the splenic reticular cells and macrophages (reticular meshwork) that described a continuum from reactive changes associated with immunologic damage of erythrocytes to neoplastic proliferation of histiocytic components. Thirty percent of the dogs survived 12 months. Approximately one half (53%) of the dogs with complete postmortem evaluations showed multiorgan involvement with a tissue distribution and cell morphology consistent with histiocytic neoplasia. For the remaining dogs (47%), only splenic pathology was consistently present, and a specific cause of death was often not evident. Distinctive histologic changes in the splenic tissues-including mitotic activity, erythrophagocytosis, giant cell formation, thrombosis/ infarction, and the proportion and distribution of histiocytic and hematopoietic cells-were statistically evaluated for prognostic relevance. The presence of giant cells was the only reliable prognostic feature, and that was indicative of a fatal outcome. These descriptive changes of myeloid metaplasia in the canine spleen are compared with the human clinical and pathologic syndromes of 1) agnogenic myeloid metaplasia, 2) hemophagocytic syndromes, and 3) hypersplenism. These diseases in humans produce histopathologic changes in the spleen that are similar to those observed in the canine splenic tissue we examined in this study.  (+info)

Neutrophil alkaline phosphatase score in chronic granulocytic leukaemia: effects of splenectomy and antileukaemic drugs. (6/532)

Staining with naphthol AS phosphate and Fast Blue BB salt has been used for the estimation of neutrophil alkaline phosphatase (NAP) scores in patients with chronic granulocytic leukaemia (CGL). The very low scores found at diagnosis rise when the disease is treated, and there is some inverse correlation between the NAP score and the absolute neutrophil count. Patients treated intensively developed high NAP scores. Elective splenectomy performed during the chronic phase of CGL is followed by a pronounced but transient neutrophilia and a concurrent striking rise in the NAP score. Similar changes were observed in patients without CGL who underwent splenectomy. These observations can be explained by assuming that newly formed neutrophils in CGL have a normal content of NAP but are rapidly sequestered in non-circulating extramedullary pools, whereas the circulating neutrophil with a typically low NAP content is a relatively aged cell which has lost enzyme activity. In subjects with or without CGL, removal of the spleen, a major site of such pooling, temporarily permits the circulation of newly formed neutrophils but eventually other organs assume the sequestering functions of the spleen. Thus the aberrations of NAP score seen in CGL might be attributable not to an intrinsic cellular defect but to an exaggeration of the granulocyte storage phenomena which also occur in subjects without CGL.  (+info)

Myeloproliferative disorders. (7/532)

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.  (+info)

Allogeneic peripheral blood cell transplantation for hypereosinophilic syndrome with myelofibrosis. (8/532)

Patients with hypereosinophilic syndrome (HES) display a very heterogeneous clinical picture ranging from asymptomatic cases to very aggressive forms. We report a 38-year-old woman with progressive HES who developed severe myelofibrosis and was treated by allogeneic stem cell transplantation, using peripheral blood (PBSCT) instead of bone marrow as the source of progenitor cells, after conditioning with cytoxan and busulphan. To the best of our knowledge, this is the first case of HES with myelofibrosis treated with PBSCT. The patient remains alive 8 months post-PBSCT, and bone marrow fibrosis has significantly decreased following transplantation. Bone Marrow Transplantation (2000) 25, 217-218.  (+info)

Agnogenic myeloid metaplasia: role of splenectomy | Postgraduate Medical JournalAgnogenic myeloid metaplasia: role of splenectomy | Postgraduate Medical Journal
The splenomegaly associated with myelofibrosis and agnogenic myeloid metaplasia should not be considered a manifestation of the fundamental proliferative process, nor should it be considered as necessarily compensatory for reduced marrow haematopoiesis.. In deserving cases splenectomy may cause an improvement in the patients general and haematopoietic status. Removal of the source of functional hypersplenism, causing haemolytic episodes and thrombocytopenia, results in marked amelioration in the clinical condition with reduction in the magnitude and frequency of replacement blood transfusion.. The massive size of the spleen associated with this condition may not only cause local pain and discomfort but may lead to traumatic or spontaneous rupture.. Consideration of two cases studied by the authors indicates that marked clinical improvement may be associated with splenectomy in selected cases of agnogenic myeloid metaplasia.. ...
http://pmj.bmj.com/content/45/522/261
A Phase 2/3 Study of Pacritinib in Patients With Primary Myelofibrosis Post Polycythemia Vera Myelofibrosis or Post-Essential...A Phase 2/3 Study of Pacritinib in Patients With Primary Myelofibrosis Post Polycythemia Vera Myelofibrosis or Post-Essential...
Clinical trial for Post-essential Thrombocythemia Myelofibrosis | Post-polycythemia Vera Myelofibrosis | Myelosclerosis with myeloid metaplasia | Myelofibrosis | Post Essential Thrombocythemia Myelofibrosis , A Phase 2/3 Study of Pacritinib in Patients With Primary Myelofibrosis Post Polycythemia Vera Myelofibrosis or Post-Essential Thrombocythemia Myelofibrosis
https://www.centerwatch.com/clinical-trials/listings/119533/primary-myelofibrosis-phase-23-study-pacritinib/?geo_lat=40.7229&geo_lng=-73.8473&radius=10&place=Forest%20Hills
Nephrotic syndrome associated with agnogenic myeloid metaplasia | AVESİSNephrotic syndrome associated with agnogenic myeloid metaplasia | AVESİS
Extramedullary hematopoiesis being an important feature of agnogenic myeloid metaplasia (AMM), a chronic myeloproliferative disease of clonal origin, may affect the kidneys, but this condition is usually asymptomatic. Until now, there is only one reported case of nephrotic syndrome associated with AMM. We present a patient with AMM who had nephrotic syndrome and whose renal biopsy revealed membranous glomerulonephritis together with renal extramedullary hematopoiesis. ...
https://avesis.istanbulc.edu.tr/yayin/a8b95577-280e-4c9d-9d46-53573a62fef5/nephrotic-syndrome-associated-with-agnogenic-myeloid-metaplasia
A Trial of Zoledronic Acid in Patients With Myelofibrosis With Myeloid Metaplasia (MMM) - Full Text View - ClinicalTrials.govA Trial of Zoledronic Acid in Patients With Myelofibrosis With Myeloid Metaplasia (MMM) - Full Text View - ClinicalTrials.gov
Inclusion Criteria:. male or female and at least 18 years-of-age histologically confirmed diagnosis of myelofibrosis with myeloid metaplasia (MMM). This includes patients with agnogenic myeloid metaplasia (also known as idiopathic myelofibrosis) and patients with a preceding history of polycythemia vera or essential thrombocytemia (also known as post-polycytemic myelofibrosis). (see Appendix A) patients with low, intermediate and high risk disease categories (following the Dupriez score) may be included presence of measurable, clinically relevant disease manifestations (especially for low risk patients) ECOG performance status of 0, 1 or 2 life expectancy of at least 3 months Women of childbearing potential must use a medically acceptable form of contraception during the study and must have a negative urine or serum pregnancy test within 7 days of randomization written informed consent. Exclusion Criteria:. diseases associated with secondary myelofibrosis, such as metastatic carcinoma, lymphoma, ...
https://clinicaltrials.gov/ct2/show/NCT00287261?cond=%22Myelofibrosis%22&rank=15
Primary Myelofibrosis: Practice Essentials, Pathophysiology, EtiologyPrimary Myelofibrosis: Practice Essentials, Pathophysiology, Etiology
Primary myelofibrosis is a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells. Older terms for this disorder include agnogenic myeloid metaplasia with myelofibrosis and chronic idiopathic myelofibrosis.
https://emedicine.medscape.com/article/197954-overview
Primary Myelofibrosis | Ask Hematologist | Understand HematologyPrimary Myelofibrosis | Ask Hematologist | Understand Hematology
References:. Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, et al. Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT). Leuk Res. 2007 Jun. 31(6):737-40.. Klampfl T, Gisslinger H, Harutyunyan AS, Nivarthi H, Rumi E, Milosevic JD, et al. Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med. 2013 Dec 19. 369(25):2379-90.. Nangalia J, Massie CE, Baxter EJ, Nice FL, Gundem G, Wedge DC, et al. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med. 2013 Dec 19. 369(25):2391-405.. Verstovsek S, Mesa RA, Gotlib J, Levy RS, Gupta V, DiPersio JF, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012 Mar 1. 366(9):799-807.. Gangat N, Caramazza D, Vaidya R, ...
https://askhematologist.com/primary-myelofibrosis/
Idiopathic myelofibrosis: dental treatment considerations.<...Idiopathic myelofibrosis: dental treatment considerations.<...
TY - JOUR. T1 - Idiopathic myelofibrosis. T2 - dental treatment considerations.. AU - Steelman, Robert. AU - Holmes, D.. AU - Cranston, R.. AU - Cupp, D.. PY - 1991/3. Y1 - 1991/3. N2 - Idiopathic myelofibrosis is a myeloproliferative disorder of unknown origin. The bone marrow becomes fibrotic with an associated decrease in hematopoiesis resulting in anemia, bleeding problems, splenomegaly, and other secondary abnormalities. Although idiopathic myelofibrosis is usually diagnosed in middle age, there have been a few reports of the disorder in the pediatric population. This case report documents dental treatment considerations in a 6-year-old female with idiopathic myelofibrosis, severe anemia, and abnormal blood coagulation studies. The patient was successfully treated in a hospital after medical consultation, transfusion of packed red blood cells, and administration of prophylactic antibiotics. Local hemostatic measures following multiple extractions of carious teeth controlled bleeding. No ...
https://ohsu.pure.elsevier.com/en/publications/idiopathic-myelofibrosis-dental-treatment-considerations-2
Activation of non-canonical TGF-β1 signaling indicates an autoimmune mechanism for bone marrow fibrosis in primary...
TY - JOUR. T1 - Activation of non-canonical TGF-β1 signaling indicates an autoimmune mechanism for bone marrow fibrosis in primary myelofibrosis. AU - Ciaffoni, Fiorella. AU - Cassella, Elena. AU - Varricchio, Lilian. AU - Massa, Margherita. AU - Barosi, Giovanni. AU - Migliaccio, Anna Rita. PY - 2015/3/1. Y1 - 2015/3/1. N2 - Primary myelofibrosis (PMF) is characterized by megakaryocyte hyperplasia, dysplasia and death with progressive reticulin/collagen fibrosis in marrow and hematopoiesis in extramedullary sites. The mechanism of fibrosis was investigated by comparing TGF-β1 signaling of marrow and spleen of patients with PMF and of non-diseased individuals. Expression of 39 (23 up-regulated and 16 down-regulated) and 38 (8 up-regulated and 30 down-regulated) TGF-β1 signaling genes was altered in the marrow and spleen of PMF patients, respectively. Abnormalities included genes of TGF-β1 signaling, cell cycling and abnormal in chronic myeloid leukemia (. EVI1 and p21CIP) (both marrow and ...
https://moh-it.pure.elsevier.com/en/publications/activation-of-non-canonical-tgf-%CE%B21-signaling-indicates-an-autoimm
Myelofibrosis Market: Latest Trends & Insights 2024 | Research Reports IndustryMyelofibrosis Market: Latest Trends & Insights 2024 | Research Reports Industry
Global Myelofibrosis Market: Overview Myelofibrosis is an uncommon type of bone marrow cancer and is related to a group of blood cancers known as myeloproliferative neoplasms. A simple blood test along with bone marrow biopsy can diagnose myelofibrosis. Myelofibrosis is also known as chronic myelosclerosis, agnogenic myeloid metaplasia, aleukemic megakaryocytic myelosis, idiopathic myelofibrosis, and leukoerythroblastosis.…
https://researchreportsindustry.wordpress.com/2017/07/18/myelofibrosis-market-latest-trends-insights-2024/
Discrepancies of applying primary myelofibrosis prognostic scores for patients with post polycythemia vera/essential...Discrepancies of applying primary myelofibrosis prognostic scores for patients with post polycythemia vera/essential...
We found agreement for risk classification was poor when DIPSS and post-PV risk scores were applied to the same post-PV/ET MF patients. Scores were calculated at fixed time interval and thus, the results are not simply representative of a change in clinical status over time. Interestingly, DIPSS was more likely to assign patients to a high-risk category than the post-PV risk assessment score.. This climate of risk prognostication has changed dramatically over the last two decades.. From the Lille4 in 1996, International Prognostic Scoring System (IPSS)5 in 2009, Dynamic International Prognostic Scoring System (DIPSS)2 in 2010, DIPSS-plus6 in 2011, to the most recent introduction of Mutation Enhanced International Prognostic Scoring System (MIPSS)7 and the Genetics-based Prognostic Scoring System (GPS)8 in 2014, accurate risk stratification within MF has been a moving target.9 To complicate the issue further, the diagnosis of secondary myelofibrosis such as in post-polycythemia vera (PV) MF and ...
http://www.haematologica.org/content/101/10/e405
Myeloproliferative Disorders and MyelofibrosisMyeloproliferative Disorders and Myelofibrosis
Ruxolitinib (Jakafi), an oral JAK1 and JAK2 kinase inhibitor, was approved in November 2011 for the treatment of patients with intermediate or high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis.14 Two phase III trials demonstrated significant improvement compared with best available therapy for spleen size, symptoms, and burden reduction, as well as for quality of life.14-16 However, the reduced spleen size was not shown to be consistently durable in a phase I/II study and neither phase III study reported a significant survival benefit.17,18 This agent is commonly associated with hematologic side effects; anemia was reported in 96% of patients taking ruxolitinib (grade 3/4, 45%), and thrombocytopenia was reported in 70% (grade 3/4, 13%).14 This first JAK inhibitor therapy for myelofibrosis has been long anticipated; yet, the value of this treatment is not truly known. The treatment of adverse events and ...
https://www.ajmc.com/journals/evidence-based-oncology/2012/2012-2-vol18-n3/myeloproliferative-disorders-and-myelofibrosis
Clinical features and outcomes of patients with primary myelofibrosis in Japan: report of a 17-year nationwide survey by the...
TY - JOUR. T1 - Clinical features and outcomes of patients with primary myelofibrosis in Japan. T2 - report of a 17-year nationwide survey by the Idiopathic Disorders of Hematopoietic Organs Research Committee of Japan. AU - Takenaka, Katsuto. AU - Shimoda, Kazuya. AU - Uchida, Naoyuki. AU - Shimomura, Taizo. AU - Nagafuji, Koji. AU - Kondo, Tadakazu. AU - Shibayama, Hirohiko. AU - Mori, Takehiko. AU - Usuki, Kensuke. AU - Azuma, Taichi. AU - Tsutsumi, Yutaka. AU - Tanaka, Junji. AU - Dairaku, Hitomi. AU - Matsuo, Keitaro. AU - Ozawa, Keiya. AU - Kurokawa, Mineo. AU - Arai, Shunya. AU - Akashi, Koichi. N1 - Publisher Copyright: © 2016, The Japanese Society of Hematology.. PY - 2017/1/1. Y1 - 2017/1/1. N2 - We conducted a 17-year nationwide survey (1999-2015) to elucidate the clinical outcomes of patients with primary myelofibrosis (PMF) in Japan. Questionnaires were sent annually to approximately 500 hematology departments. Newly diagnosed patients with PMF were enrolled in this study, and were ...
https://kyushu-u.pure.elsevier.com/ja/publications/clinical-features-and-outcomes-of-patients-with-primary-myelofibr
Phase I Study of GC1008 in Patients With Primary Myelofibrosis (PMF), Post-polycythemia Vera/Essential Thrombocythemia Related...Phase I Study of GC1008 in Patients With Primary Myelofibrosis (PMF), Post-polycythemia Vera/Essential Thrombocythemia Related...
This trial will assess the tolerability and efficacy of fresolimumab (GC1008, monoclonal antibody to TGF-beta) in patients with primary myelofibrosis or
http://adisinsight.springer.com/trials/700194470?error=cookies_not_supported&code=e23cb448-88b5-4fb3-8f06-aa298111f364
A longitudinal study of the JAK2<sup>V617F</sup> mutation in myelofibrosis with myeloid metaplasia: Analysis at two time...
TY - JOUR. T1 - A longitudinal study of the JAK2V617F mutation in myelofibrosis with myeloid metaplasia. T2 - Analysis at two time points. AU - Mesa, Ruben A.. AU - Powell, Heather. AU - Lasho, Terra. AU - DeWald, Goron W.. AU - McClure, Rebecca. AU - Tefferi, Ayalew. PY - 2006/3/1. Y1 - 2006/3/1. N2 - Serial analysis for the activating JAK2V617F mutation performed in 44 patients with myelofibrosis with myeloid metaplasia showed no interval change in 88% (22/25) of patients over a median interval of 18.6 months. The increase in JAK2 expression observed in three patients did not correspond to disease progression or leukemic transformation.. AB - Serial analysis for the activating JAK2V617F mutation performed in 44 patients with myelofibrosis with myeloid metaplasia showed no interval change in 88% (22/25) of patients over a median interval of 18.6 months. The increase in JAK2 expression observed in three patients did not correspond to disease progression or leukemic transformation.. KW - ...
https://mayoclinic.pure.elsevier.com/en/publications/a-longitudinal-study-of-the-jak2supv617fsup-mutation-in-myelofibr
Definition of agnogenic myeloid metaplasia - NCI Dictionary of Cancer Terms - National Cancer InstituteDefinition of agnogenic myeloid metaplasia - NCI Dictionary of Cancer Terms - National Cancer Institute
A progressive, chronic disease in which the bone marrow is replaced by fibrous tissue and blood is made in organs such as the liver and the spleen, instead of in the bone marrow. This disease is marked by an enlarged spleen and progressive anemia.
https://www.cancer.gov/publications/dictionaries/cancer-terms/def/agnogenic-myeloid-metaplasia
A Phase 1/2 Study of Oral SB1518 in Subjects With Chronic Idiopathic Myelofibrosis - Full Text View - ClinicalTrials.govA Phase 1/2 Study of Oral SB1518 in Subjects With Chronic Idiopathic Myelofibrosis - Full Text View - ClinicalTrials.gov
The study consists of two phases: The first portion of the study is a Phase 1 dose escalation study to determine the maximum tolerated dose and the dose limiting toxicities of SB1518 when given as a single agent orally once daily in subjects with Chronic Idiopathic Myelofibrosis (CIMF) regardless of their JAK2 mutational status. The second portion of the study is a Phase 2 study to define the efficacy and safety profile of single agent SB1518 at the recommended dose in subjects with CIMF ...
https://clinicaltrials.gov/ct2/show/NCT00745550
The impact of anemia on overall survival in patients with myelofibrosis treated with ruxolitinib in the COMFORT studies |...The impact of anemia on overall survival in patients with myelofibrosis treated with ruxolitinib in the COMFORT studies |...
Key words. Myelofibrosis (MF), including primary myelofibrosis (PMF) and MF secondary to essential thrombocythemia (ET) or polycythemia vera (PV), is a chronic Philadelphia chromosome-negative myeloproliferative neoplasm associated with progressive bone marrow fibrosis.1 Many patients with MF experience new or worsening anemia during disease progression. Varying from study to study, 35% to 54% of patients with PMF have been reported to have anemia (i.e., hemoglobin ,10 g/dL) at the time of diagnosis.2-5 Anemia adversely affects overall survival (OS), and is included as a key negative prognostic factor in validated prognostic scoring systems for patients with PMF, which were developed before the introduction of Janus kinase (JAK) inhibitor therapy.2,3,5 Ruxolitinib, a JAK1/JAK2 inhibitor, improved OS compared with placebo and best available therapy in patients with intermediate-2 or high-risk MF5 in the phase 3 COntrolled MyeloFibrosis Study With ORal JAK Inhibitor Treatment (COMFORT) ...
http://www.haematologica.org/content/101/12/e482
Bolder Science | NCT03755518Bolder Science | NCT03755518
Brief summary:. This is Single-Arm, Open-Label Efficacy and Safety Trial of Fedratinib in Subjects with DIPSS (Dynamic International Prognostic Scoring System)-Intermediate or High- Risk Primary Myelofibrosis (PMF), Post-Polycythemia Vera Myelofibrosis (post-PV MF), or Post-Essential Thrombocythemia Myelofibrosis (post-ET MF) and Previously Treated with Ruxolitinib. The primary objective of the study is to evaluate the percentage of subjects with at least a 35% reduction of spleen volume and one of the secondary objectives is to evaluate the safety of fedratinib ...
https://www.bolderscience.com/trial/nct03755518/
Post-Polycythemia Vera Myelofibrosis (PPV-MF) - PipelinePost-Polycythemia Vera Myelofibrosis (PPV-MF) - Pipeline
Press release - ReportsWorldwide - Post-Polycythemia Vera Myelofibrosis (PPV-MF) - Pipeline Review, H1 2017 - published on openPR.com
https://www.openpr.com/news/566681/post-polycythemia-vera-myelofibrosis-ppv-mf-pipeline-review-h1-2017.html
Chronic Idiopathic Myelofibrosis Research Report - Forecast 2017 - 2025 | CultHub
New York, NY -- 01/12/2018 -- Idiopathic myelofibrosis is a chronic myelo-proliferative disorder and characterized by abnormal mutation of stem cells. This abnormal mutation of stem cells and excessive production of platelets result in development of fibrous tissues within the bone-marrow. This factor would ultimately negatively affect on the development of white blood cells (WBCs),…
https://culthub.com/entertainment/chronic-idiopathic-myelofibrosis-research-report-forecast-2017-2025/58937
CALR and ASXL1 mutations-based molecular prognostication in primary myelofibrosis: An international study of 570 patients<...
TY - JOUR. T1 - CALR and ASXL1 mutations-based molecular prognostication in primary myelofibrosis. T2 - An international study of 570 patients. AU - Tefferi, A.. AU - Guglielmelli, P.. AU - Lasho, T. L.. AU - Rotunno, G.. AU - Finke, C.. AU - Mannarelli, C.. AU - Belachew, A. A.. AU - Pancrazzi, A.. AU - Wassie, E. A.. AU - Ketterling, R. P.. AU - Hanson, C. A.. AU - Pardanani, A.. AU - Vannucchi, A. M.. N1 - Funding Information: This study was supported by the Mayo Clinic Harvey-Yulman Charitable Foundation for Myelofibrosis Tissue Bank and Clinical Database of Molecular and Biological Abnormalities and by a special grant from Associazione Italiana per la Ricerca sul Cancro-AIRC 5 per Mille-to AGIMM, AIRC-Gruppo Italiano Malattie Mieloprolifera-tive (no. 1005) to AMV; for a description of the AGIMM project, see at http:// www.progettoagimm.it). Partially supported by Ministero della Università e Ricerca (MIUR; FIRB project #RBAP11CZLK and PRIN 2010NYKNS7 to AMV).. PY - 2014/7. Y1 - ...
https://mayoclinic.pure.elsevier.com/en/publications/calr-and-asxl1-mutations-based-molecular-prognostication-in-prima
Aging | Transcriptome profiling reveals target in primary myelofibrosis together with structural biology study on novel natural...
This study aimed to identify effective targets for carcinogenesis of primary myelofibrosis (PMF), as well as to screen ideal lead compounds with potential inhibition effect on Janus kinase 2 to contribute to the medication design and development. Gene expression profiles of GSE26049, GSE53482, GSE61629 were obtained from the Gene Expression Omnibus database. The differentially expressed genes were identified, and functional enrichment analyses such as Gene Ontology, protein-protein interaction network etc., were performed step by step. Subsequently, highly-precise computational techniques were conducted to identify potential inhibitors of JAK2. A series of structural biology methods including virtual screening, ADMET (absorption, distribution, metabolism, excretion, and toxicity) prediction, molecule docking, molecular dynamics simulation etc., were implemented to discover novel natural compounds. Results elucidated that PMF patients had abnormal LCN2, JAK2, MMP8, CAMP, DEFA4, LTF, MPO, HBD, STAT4, EBF1
https://www.aging-us.com/figure/202635/f2
Fedratinib May Represent New Option for Jakafi-Resistant Myelofibrosis « Pacific cancer care
Patients with myelofibrosis resistant or intolerant to Jakafi (ruxolitinib) may have an alternative treatment option with a novel JAK2-selective inhibitor fedratinib, according to the results of clinical study recently published in the medical journal Lancet.1. About Myelofibrosis. Myelofibrosis is a type of blood cancer known as a myeloproliferative neoplasm that is chronic and progressive in nature. It involves the abnormal development and function of bone marrow cells that produce blood cells and leads to the formation of scar tissue in the bone marrow. When the bone marrow becomes scarred it cant make enough blood cells and this can cause anemia, enlargement of the spleen and liver, fatigue, and other problems.. Myelofibrosis can result from a worsening of other bone marrow diseases, such as polycythemia vera and essential thrombocythemia or develop on its own - so called primary myelofibrosis.. Approved in 2011, Jakafi is currently the only drug that has been approved specifically for ...
http://pacificcancercare.com/2017/12/06/fedratinib-may-represent-new-option-for-jakafi-resistant-myelofibrosis/
Idiopathic Myelofibrosis in an Infant.
Idiopathic Myelofibrosis (MF) is an extremely rare condition in children. It has a very variable clinical spectrum. Cases of secondary myelofibrosis associated with Vitamin D deficiency and Systemic Lupus Erythematosus have been reported from India .
http://www.biomedsearch.com/nih/Idiopathic-Myelofibrosis-in-Infant/21188553.html
Efficacy and safety of ruxolitinib in Asian patients with myelofibrosis<...
TY - JOUR. T1 - Efficacy and safety of ruxolitinib in Asian patients with myelofibrosis. AU - Jung, Chul Won. AU - Shih, Lee Yung. AU - Xiao, Zhijian. AU - Jie, Jin. AU - Hou, Hsin An. AU - Du, Xin. AU - Wang, Ming Chung. AU - Park, Seonyang. AU - Eom, Ki Seong. AU - Oritani, Kenji. AU - Okamoto, Shinichiro. AU - Tauchi, Tetsuzo. AU - Kim, Jin Seok. AU - Zhou, Daobin. AU - Saito, Shigeki. AU - Li, Junmin. AU - Handa, Hiroshi. AU - Jianyong, Li. AU - Ohishi, Kohshi. AU - Hou, Ming. AU - Depei, Wu. AU - Takenaka, Katsuto. AU - Liu, Ting. AU - Hu, Yu. AU - Amagasaki, Taro. AU - Ito, Kazuo. AU - Gopalakrishna, Prashanth. AU - Akashi, Koichi. PY - 2015/7/1. Y1 - 2015/7/1. N2 - Myelofibrosis is characterized by progressive cytopenias, bone marrow fibrosis, splenomegaly and severe constitutional symptoms. In the phase 3 Controlled Myelofibrosis Study with Oral JAK Inhibitor Treatment (COMFORT) studies, ruxolitinib, a potent Janus kinase 1 (JAK1)/JAK2 inhibitor, provided substantial improvements in ...
https://kyushu-u.pure.elsevier.com/en/publications/efficacy-and-safety-of-ruxolitinib-in-asian-patients-with-myelofi
Primary Myelofibrosis Prognosis Tool - WHO Criteria for PMF DiagnosisPrimary Myelofibrosis Prognosis Tool - WHO Criteria for PMF Diagnosis
The primary myelofibrosis prognosis tool helps evaluate whether a patients diagnosis is consistent with the World Health Organization (WHO) criteria for a diagnosis of PMF.
https://www.mpnconnect.com/primary-myelofibrosis-prognosis-tool.aspx
Internuclear bridging of erythroid precursors in the peripheral blood smear in a patient with primary myelofibrosis [Turk J...
Roger K. Schindhelm, Marije M. Van Santen, Arie C. Van Der Spek. Internuclear bridging of erythroid precursors in the peripheral blood smear in a patient with primary myelofibrosis. Turk J Hematol. 2017; 34(1): 124- ...
http://aoh.tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-40360
JAK Inhibitor Provides Rapid, Durable Relief for Myelofibrosis Patients | MD Anderson Cancer CenterJAK Inhibitor Provides Rapid, Durable Relief for Myelofibrosis Patients | MD Anderson Cancer Center
MD Anderson News Release 09/15/2010. Life-threatening bone marrow malignancy has no approved therapy. MD Anderson News Release 09/15/10. An oral medication produces significant and lasting relief for patients with myelofibrosis, a debilitating and lethal bone marrow disorder, researchers at The University of Texas MD Anderson Cancer Center report in the Sept. 16 New England Journal of Medicine.. Myelofibrosis is caused by the accumulation of malignant bone marrow cells that trigger an inflammatory response, scarring the bone marrow and limiting its ability to produce blood, causing anemia.. The problem with myelofibrosis is the lack of available therapies for patients - there are none approved for this disease today, said principal investigator Srdan Verstovsek, M.D., Ph.D., associate professor in MD Andersons Department of Leukemia. Average life expectancy for people with this disease is 5 to 7 years. Available therapies approved for other diseases provide little response and are mainly ...
https://www.mdanderson.org/newsroom/2010/09/jak-inhibitor-provides-rapid-durable-relief-for-myelofibrosis-pa.html
FDA Approves Inrebic Capsules for Myelofibrosis | Doctors LoungeFDA Approves Inrebic Capsules for Myelofibrosis | Doctors Lounge
FRIDAY, Aug. 16, 2019 (HealthDay News) -- Inrebic (fedratinib) capsules have been approved to treat adults with intermediate-2 or high-risk primary or secondary myelofibrosis, making it the second drug approved to treat patients with this disease, the U.S. Food and Drug Administration announced today.. Approval of Inrebic for patients with intermediate-2 or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis was based on clinical trial data from 289 patients randomly assigned to 400 or 500 mg of oral Inrebic daily or placebo. Thirty-six percent of patients (35 of 96) treated with the label-recommended dose of 400 mg of Inrebic had experienced at least a 35 percent reduction in spleen volume from baseline to week 24 as measured by magnetic resonance imaging or computed tomography scan. Thirty-six patients treated with Inrebic had at least a 50 percent reduction in myelofibrosis-related symptoms, including night sweats, itching, abdominal ...
https://www.doctorslounge.com/index.php/news/hd/90577
Stem cell origin of human myeloid blood cell neoplasms. - Semantic ScholarStem cell origin of human myeloid blood cell neoplasms. - Semantic Scholar
Studies with G6PD and molecular probes indicate that the myeloid leukemias and the chronic myeloproliferative disorders are clonal diseases. The G6PD data indicate that chronic myelogenous leukemia, polycythemia vera and essential thrombocythemia involve stem cells pluripotent for granulocytes, erythrocytes, megakaryocytes and lymphocytes. Agnogenic myeloid metaplasia is also a clonal disease that involves multipotent hematopoietic stem cells. However, myelofibrosis, the predominant clinical manifestation, occurs secondarily and is not a component of the abnormal clonal proliferation. Acute nonlymphocytic leukemia is a clonal disease, but G6PD studies suggest that there are at least two forms of this leukemia. In one type of ANL, the involved stem cells exhibit pluripotent differentiative expression. In another type of ANL, differentiative expression is largely restricted to the granulocytic pathway. The heterogeneity of ANL has both clinical and pathogenetic implications.
https://www.semanticscholar.org/paper/Stem-cell-origin-of-human-myeloid-blood-cell-neopl-Fialkow/7094f12664c068256a0931e3adf109ac97bf3286
Adenine Receptors - Angiogenesis Inhibitors ResearchAdenine Receptors - Angiogenesis Inhibitors Research
Splenomegaly is a common indication of primary myelofibrosis (PMF), post-polycythemia vera myelofibrosis (post-PV MF), and post-essential thrombocythemia myelofibrosis (post-ET MF) thats connected with bothersome symptoms, that have a significant bad impact on sufferers standard of living. JAK1/JAK2) inhibitors for the treating sufferers with ET, PV, and MF. A few of these studies have noted significant clinical advantage of JAK inhibitors, especially with regards to regression of splenomegaly. PIK3CA In November 2011, the united states Food and Medication Administration approved the usage of the JAK1- and JAK2-selective inhibitor ruxolitinib for the treating sufferers with intermediate or high-risk myelofibrosis, including PMF, post-PV MF, and post-ET MF. This review discusses current healing choices for splenomegaly connected with principal or supplementary MF and the procedure potential from the JAK inhibitors within this placing. reported the outcomes of a stage II trial with low-dose ...
http://angiogenesis-blog.com/category/adenine-receptors/
Myelofibrosis with Myeloid Metaplasia in Survivors of the Atomic Bomb in Hiroshima | Annals of Internal Medicine | American...Myelofibrosis with Myeloid Metaplasia in Survivors of the Atomic Bomb in Hiroshima | Annals of Internal Medicine | American...
Idiopathic generalized myelofibrosis with myeloid metaplasia is a disease of unknown etiology in which the clinical and morphologic manifestations vary greatly even during the course of an individual case. The resulting confusion has led to an undue number of synonyms, each of which tends to focus on a single aspect of the disease. Many cases exhibit panhyperplasia of all bone marrow elements including fibroblasts, although eventually the more characteristic picture of fibrosis may predominate. Myeloid metaplasia is a constant and persistent finding, even when the bone marrow is hypercellular. A leukoerythroblastic peripheral blood picture is usually present at some time ...
http://annals.org/aim/article-abstract/679004/myelofibrosis-myeloid-metaplasia-survivors-atomic-bomb-hiroshima
Erythropoietin Therapy Does Not Benefit Transfusion-Dependent Primary Myelofibrosis Patients and Treatment Response Is...Erythropoietin Therapy Does Not Benefit Transfusion-Dependent Primary Myelofibrosis Patients and Treatment Response Is...
Abstract. BACKGROUND: The potential risks of tumor growth promotion and thromboembolism associated with erythropoietin (Epo) therapy warrant cautious use of er
https://ashpublications.org/blood/article/110/11/3555/58087/Erythropoietin-Therapy-Does-Not-Benefit
Conventional and experimental drug therapy in myelofibrosis with myeloid metaplasiaConventional and experimental drug therapy in myelofibrosis with myeloid metaplasia
Myelofibrosis with myeloid metaplasia (MMM) is currently classified as a classic (ie, BCR-ABL-negative) myeloproliferative disorder characterized by anemia, multiorgan extramedullary hematopoiesis, constitutional symptoms, and premature death from either leukemic transformation or other disease comp …
https://pubmed.ncbi.nlm.nih.gov/20425385/
Role of neoplastic monocyte-derived fibrocytes in primary myelofibrosis | JEMRole of neoplastic monocyte-derived fibrocytes in primary myelofibrosis | JEM
BM fibrosis in PMF is presumed to be caused by growth factors released from clonal megakaryocytes or platelets that stimulate MSCs to induce BM fibrosis (Groopman, 1980). Here, we demonstrate that clonal neoplastic fibrocytes, which are significantly expanded in patients with PMF, are functionally distinct from normal fibrocytes (perhaps because of constitutive JAK2 signaling) and contribute to the formation of BM fibrosis. Furthermore, SAP (PRM-151) slowed the development of fibrosis and significantly improved survival of NSG mice transplanted with PMF BM cells.. That fibrocytes play a direct role in the induction of BM fibrosis is not unprecedented. Ohishi et al. (2012) found an expanded population of CD45+ cells that produced type I collagen (fibrocytes) in the BM of mice conditionally expressing active parathyroid hormone receptor, results that are reminiscent of our data obtained from the mouse MPL-W515L-induced PMF model. In the parathyroid hormone receptor mice, the numbers of MSCs in BM ...
http://jem.rupress.org/content/early/2016/07/26/jem.20160283
Expression of Jak2V617F causes a polycythemia vera-like disease with associated myelofibrosis in a murine bone marrow...Expression of Jak2V617F causes a polycythemia vera-like disease with associated myelofibrosis in a murine bone marrow...
An acquired somatic mutation, Jak2V617F, was recently discovered in most patients with polycythemia vera (PV), chronic idiopathic myelofibrosis (CIMF), and essential thrombocythemia (ET). To investigate the role of this mutation in vivo, we transplanted bone marrow (BM) transduced with a retrovirus expressing either Jak2 wild-type (wt) or Jak2V617F into lethally irradiated syngeneic recipient mice. Expression of Jak2V617F, but not Jak2wt, resulted in clinicopathologic features that closely resembled PV in humans. These included striking elevation in hemoglobin level/hematocrit, leukocytosis, megakaryocyte hyperplasia, extramedullary hematopoiesis resulting in splenomegaly, and reticulin fibrosis in the bone marrow. Histopathologic and flow cytometric analyses showed an increase in maturing myeloid lineage progenitors, although megakaryocytes showed decreased polyploidization and staining for acetylcholinesterase. In vitro analysis of primary cells showed constitutive activation of Stat5 and cytokine
https://www.semanticscholar.org/paper/Expression-of-Jak2V617F-causes-a-polycythemia-vera-Wernig-Mercher/771ff99d778d1f925441598c887570ab6284158e
Myelofibrosis - Symptoms and causes - Mayo ClinicMyelofibrosis - Symptoms and causes - Mayo Clinic
Myelofibrosis - Comprehensive overview covers diagnosis and treatments, including bone marrow transplant, for myelofibrosis and primary myelofibrosis.
https://www.mayoclinic.org/diseases-conditions/myelofibrosis/symptoms-causes/syc-20355057?reDate=27072016
Interim analysis of safety and efficacy of ruxolitinib in patients with myelofibrosis and low platelet counts<...
TY - JOUR. T1 - Interim analysis of safety and efficacy of ruxolitinib in patients with myelofibrosis and low platelet counts. AU - Talpaz, Moshe. AU - Paquette, Ronald. AU - Afrin, Lawrence. AU - Hamburg, Solomon I.. AU - Prchal, Josef T.. AU - Jamieson, Katarzyna. AU - Terebelo, Howard R.. AU - Ortega, Gregory L.. AU - Lyons, Roger M.. AU - Tiu, Ramon V.. AU - Winton, Elliott F.. AU - Natrajan, Kavita. AU - Odenike, Olatoyosi. AU - Claxton, David. AU - Peng, Wei. AU - ONeill, Peter. AU - Erickson-Viitanen, Susan. AU - Leopold, Lance. AU - Sandor, Victor. AU - Levy, Richard S.. AU - Kantarjian, Hagop M.. AU - Verstovsek, Srdan. PY - 2013/10/31. Y1 - 2013/10/31. N2 - Background: Ruxolitinib, a Janus kinase 1 and 2 inhibitor, demonstrated improvements in spleen volume, symptoms, and survival over placebo and best available therapy in intermediate-2 or high-risk myelofibrosis patients with baseline platelet counts ≥100 × 109/L in phase III studies. The most common adverse events were ...
https://augusta.pure.elsevier.com/en/publications/interim-analysis-of-safety-and-efficacy-of-ruxolitinib-in-patient
Protein Inhibitor INCB039110 Appears Active in MyelofibrosisProtein Inhibitor INCB039110 Appears Active in Myelofibrosis
CancerConnect News: In early analysis from a Phase II clinical trial, the JAK1 inhibitor INCB039110 appears to improve symptoms in patients with myelofibrosis. These findings were presented at the 56th American Hematological Society Annual Meeting and Exposition, December 6-9, 2014, in San Francisco, California.. Myelofibrosis is a type of blood cancer known as a myeloproliferative neoplasm. It involves the abnormal development and function of bone marrow cells that produce blood cells and leads to the formation of scar tissue in the bone marrow. This can cause anemia, enlargement of the spleen and liver, fatigue, and other problems. In some patients with myelofibrosis, the condition progresses to acute myeloid leukemia.. Proteins known as JAK1 and JAK2 may play a role in the development of MPNs, including myelofibrosis, by causing the body to make the wrong number of blood cells. Drugs that suppress JAK1 and JAK2 are used to treat different forms of MPN by reducing the number of abnormal number ...
http://news.cancerconnect.com/protein-inhibitor-incb039110-appears-active-in-myelofibrosis/
Ruxolitinib Treatment in a Patient with Primary Myelofibrosis Resistant to Conventional Therapies and Splenectomy: A Case...
D rt y l nce primer myelofibrozis tan s konulan 67 ya ndaki erkek hastaya uygulanan konvansiyonel tedavi y ntemleri ile sonu al namad . Dalak boyutlar ileri derecede artt , hastan n tekrar ayda 4-6 nite transf zyon gereksinimi olmaya ba lad . Bu d nemde dev boyutlara ula an dalakta infarkt s geli ti ve hastaya splenektomi yapt r ld . Splenektomi sonras hastaya ruxolitinib ba land . Ruxolitinib tedavisinin 1. ay ndan itibaren hasta transf zyon ba ms z hale geldi, t m konstit syonel semptomlar ortadan kalkt . Ancak ruxolitinib tedavisinin 6. ay nda hasta akut myeloblastik l semiye (AML) transfore oldu. Ve AML tedavisinin 1. ay nda hasta kaybedildi. Bu olgu splenektomi yap lm bir hastada ruxolitinib etkisini g steren ilk olgudur.. Anahtar Kelimeler: Primer myelofibrozis, Ruxolitinib, ...
http://aoh.tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-70370&look4=
Ruxolitinib in combination with lenalidomide as therapy for patients with myelofibrosis<...
TY - JOUR. T1 - Ruxolitinib in combination with lenalidomide as therapy for patients with myelofibrosis. AU - Daver, Naval. AU - Cortes, Jorge. AU - Newberry, Kate. AU - Jabbour, Elias. AU - Zhou, Lingsha. AU - Wang, Xuemei. AU - Pierce, Sherry. AU - Kadia, Tapan. AU - Sasaki, Koji. AU - Borthakur, Gautam. AU - Ravandi, Farhad. AU - Pemmaraju, Naveen. AU - Kantarjian, Hagop. AU - Verstovsek, Srdan. PY - 2015/8/5. Y1 - 2015/8/5. N2 - Ruxolitinib and lenalidomide may target distinct clinical and pathological manifestations of myelofibrosis and prevent therapy-related worsening of blood cell counts. To determine the efficacy and safety of the combination in patients with myelofibrosis, patients were given 15 mg ruxolitinib orally twice daily in continuous 28-day cycles, plus 5 mg lenalidomide orally once daily on days 1-21. Thirty-one patients were treated, with a median followup of 28 months (range, 12 - 35+). Due to failure to meet the predetermined efficacy rules for treatment success the study ...
https://augusta.pure.elsevier.com/en/publications/ruxolitinib-in-combination-with-lenalidomide-as-therapy-for-patie
Unmet Demands Create Opportunity for Blockbuster Products for Treating Myelofibrosis | Mar 8, 2017 - ReleaseWireUnmet Demands Create Opportunity for Blockbuster Products for Treating Myelofibrosis | Mar 8, 2017 - ReleaseWire
Press Release issued Mar 8, 2017: MarketResearchReports.biz has added a new market study to its repository, titled Opportunity Analyzer: Myelofibrosis - Opportunity Analysis And Forecasts To 2025. Myelofibrosis (MF) is a rare and serious blood disorder, which is characterized by bone marrow fibrosis. It hampers the bodys normal production of blood cells. At present, there is just one approved drug, Incyte/Novartis Jakafi (ruxolitinib), for the treatment of MF, and other conventional therapies leveraged to treat MF are off-label. Some of the off-label therapies are cytoreductive drug, androgen therapies, erythropoiesis-stimulating agents, immunomodulatory imide drugs, and anti-fibrotic agents.
http://www.sbwire.com/press-releases/unmet-demands-create-opportunity-for-blockbuster-products-for-treating-myelofibrosis-779336.htm
Primary Myelofibrosis Prevelance in 8 Major Markets 2016-2026 with Overview of Risk Factors, Disease Diagnosis and Prognosis --...Primary Myelofibrosis Prevelance in 8 Major Markets 2016-2026 with Overview of Risk Factors, Disease Diagnosis and Prognosis --...
/PRNewswire/ -- Research and Markets has announced the addition of the Primary Myelofibrosis Forecast in 8 Major Markets 2016-2026 report to their offering....
https://www.prnewswire.co.uk/news-releases/primary-myelofibrosis-prevelance-in-8-major-markets-2016-2026-with-overview-of-risk-factors-disease-diagnosis-and-prognosis----research-and-markets-585421621.html
Coexistence of Plasma Cell Dyscrasia with Prefibrotic Stage of Primary Myelofibrosis: A Case ReportCoexistence of Plasma Cell Dyscrasia with Prefibrotic Stage of Primary Myelofibrosis: A Case Report
International Scholarly Research Notices is a peer-reviewed, Open Access journal covering a wide range of subjects in science, technology, and medicine. The journals Editorial Board as well as its Table of Contents are divided into 108 subject areas that are covered within the journals scope.
https://www.hindawi.com/journals/isrn/2011/404057/ref/
Bone marrow fibrosis and diagnosis of essential thrombocythemiaBone marrow fibrosis and diagnosis of essential thrombocythemia
2009 (English)In: Journal of Clinical Oncology, ISSN 0732-183X, E-ISSN 1527-7755, Vol. 27, no 34, e220-1; author reply e222 p.Article in journal, Letter (Refereed) Published ...
http://uu.diva-portal.org/smash/record.jsf?pid=diva2:317659
Main myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the - DNA Damage Signalling and Repair...Main myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the - DNA Damage Signalling and Repair...
Main myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the spleen and liver as well as a variety of additional organs. biopsy exposed a hypercellular marrow moderately improved reticulin fibrosis and features consistent with main myelofibrosis. Abdominal imaging showed a normal-size spleen and did not determine any sites of EMH outside of the liver. The analysis of myelofibrosis was therefore made and this case shown predominant tropism to a transplanted freebase liver graft with absence of EMH elsewhere. We would therefore like to emphasize that findings of EMH in subjects with no preexisting hematologic neoplasm should warrant close follow-up and assessment. 1 Introduction Classified like a BCR-ABL bad myeloproliferative neoplasm [1] myelofibrosis is definitely a clonal cell malignancy characterized by progressive bone marrow fibrosis and ineffective erythropoiesis [2]. Extramedullary hematopoiesis is definitely a well-recognized trend of this disease process. ...
https://ranscombehouseglynde.com/main-myelofibrosis-pmf-commonly-results-in-extramedullary-hematopoiesis-emh-in-the/
Effect of 2-chlorodeoxyadenosine therapy on bone marrow fibrosis in hairy cell leukemiaEffect of 2-chlorodeoxyadenosine therapy on bone marrow fibrosis in hairy cell leukemia
Detail záznamu - Effect of 2-chlorodeoxyadenosine therapy on bone marrow fibrosis in hairy cell leukemia - Detailné zobrazenie záznamu - Slovenská lekárska knižnica
https://arl4.library.sk/arl-sllk/sk/detail-sllk_un_cat-r025406-Effect-of-2chlorodeoxyadenosine-therapy-on-bone-marrow-fibrosis-in-hairy-cell-leukemia/
CTI BioPharma Initiates Rolling Submission of New Drug Application (NDA) for Pacritinib in Myelofibrosis Patients with Severe...CTI BioPharma Initiates Rolling Submission of New Drug Application (NDA) for Pacritinib in Myelofibrosis Patients with Severe...
CTI BioPharma Initiates Rolling Submission of New Drug Application (NDA) for Pacritinib in Myelofibrosis Patients with Severe Thrombocytopenia - read this article along with other careers information, tips and advice on BioSpace
https://www.biospace.com/article/releases/cti-biopharma-initiates-rolling-submission-of-new-drug-application-nda-for-pacritinib-in-myelofibrosis-patients-with-severe-thrombocytopenia/
Ruxolitinib and interferon-α2 combination therapy for patients with polycythemia vera or myelofibrosis - Research -...
We report the final two-year end-of-study results from the first clinical trial investigating combination treatment with ruxolitinib and low-dose pegylated interferon-α2 (PEG-IFNα2). The study included 32 patients with polycythemia vera (PV) and 18 with primary- or secondary myelofibrosis (MF); 46 patients were previously intolerant or refractory to PEG-IFNα2. The primary outcome was efficacy, based on hematological parameters, quality of life measurements, and JAK2 V617F allele burden. We used the 2013 ELN and IWG-MRT response criteria, including response in symptoms, splenomegaly, peripheral blood counts, and bone marrow. Of 32 patients with PV, 10 (31%) achieved remission; 3 (9%) achieved complete remission. Of 18 patients with MF, 8 (44%) achieved remission; 5 (28%) achieved complete remission. The cumulative incidence of peripheral blood count remission was 0.85 and 0.75 for patients with PV and MF, respectively. MPN-SAF total symptom score decreased from 22 (95%CI, 16-29) at baseline to ...
https://research.regionh.dk/rigshospitalet/en/publications/ruxolitinib-and-interferon2-combination-therapy-for-patients-with-polycythemia-vera-or-myelofibrosis
Magnitude of Thrombosis Risk in Patients With Myeloproliferative Neoplasms | Annals of Internal Medicine | American College of...Magnitude of Thrombosis Risk in Patients With Myeloproliferative Neoplasms | Annals of Internal Medicine | American College of...
Myeloproliferative diseases were first described by William Dameshek in 1951. In 2008, the World Health Organization established a new classification system and introduced the term myeloproliferative neoplasms (MPNs). Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are the most prevalent MPNs and are characterized by overproduction of leukocytes, erythrocytes, or platelets; development of bone marrow fibrosis; leukemic transformation; and arterial and venous thrombosis. When Dameshek proposed the term myeloproliferative diseases, he also proposed the presence of a then-undiscovered stimulus that drove proliferation. We now understand that mutation of the JAK2 gene, JAK2 V617F, is the most common stimulus, occurring in 95% of patients with PV and 60% of those with ET or PMF. Myeloproliferative neoplasms are relatively rare; are acquired in middle to older age; and are, despite their classification as neoplasms, indolent diseases, with survival measured ...
http://annals.org/aim/article-abstract/2670030/shedding-new-light-magnitude-thrombosis-risk-patients-myeloproliferative-neoplasms
Primary autoimmune myelofibrosis: a case report and review of the literature<...
TY - JOUR. T1 - Primary autoimmune myelofibrosis. T2 - a case report and review of the literature. AU - Abaza, Yasmin. AU - Yin, C. Cameron. AU - Bueso-Ramos, Carlos E.. AU - Wang, Sa A.. AU - Verstovsek, Srdan. N1 - Publisher Copyright: © 2016, The Japanese Society of Hematology. Copyright: Copyright 2017 Elsevier B.V., All rights reserved.. PY - 2017/4/1. Y1 - 2017/4/1. N2 - Autoimmune myelofibrosis is a rare, distinct clinicopathological entity that can occur in isolation (primary) or in association with systemic autoimmune disorders (secondary), such as systemic lupus erythematosus and Sjogrens syndrome. This disease is characterized by isolated or combined chronic cytopenias associated with autoimmune phenomena and bone-marrow fibrosis. Due to the rarity of this disease, patients are frequently misdiagnosed as having primary myelofibrosis, the most common form of bone-marrow fibrosis. Distinguishing between both disease entities is essential given the drastic therapeutic and prognostic ...
https://www.scholars.northwestern.edu/en/publications/primary-autoimmune-myelofibrosis-a-case-report-and-review-of-the-
Inflammatory picture of Philadelphia-negative myeloproliferative neoplasms | Hematology, Transfusion and Cell Therapy
In this issue of the Hematology, Transfusion and Cell Therapy Journal, Cacemiro et al. evaluated the plasma cytokine profile of 47 patients with Ph-negative myeloproliferative neoplasms (MPN) [essential thrombocythemia (ET), primary myelofibrosis (PMF), and polycythemia vera (PV)] and of healthy subjects.1 They demonstrated increased levels of pro-inflammatory cytokines in MPN patients and higher levels of interferon (IFN)-γ-induced protein 10 (IP-10) in PMF patients with the JAK2 V617F mutation. They found differences in the cytokine profile among the three MPN disorders, including increased levels of IL-12p70, IL-17A, and RANTES in PMF, showing that MPN, in particular PMF, have altered inflammatory profiles. However, their sample population did not make clinical and prognostic implications of their findings possible.. What is the clinical relevance of the altered cytokine levels in MPN? Are they related to constitutional symptoms, transformation or evolution to fibrosis? Do they have an ...
http://www.htct.com.br/en-inflammatory-picture-philadelphia-negative-myeloproliferative-neoplasms-articulo-S253113791830052X
ANALYTE DETECTION USING A NEEDLE PROJECTION PATCH - Patent applicationANALYTE DETECTION USING A NEEDLE PROJECTION PATCH - Patent application
0339] In still other embodiments, polypeptide analytes are selected from antigens including endogenous antigens produced by a host or exogenous antigens that are foreign to that host. The antigens may be in the form of soluble peptides or polypeptides or polynucleotides from which an expression product (e.g., protein or RNA) is producible. Suitable endogenous antigens include, but are not restricted to, cancer or tumor antigens. Non-limiting examples of cancer or tumor antigens include antigens from a cancer or tumor selected from ABL1 proto-oncogene, AIDS related cancers, acoustic neuroma, acute lymphocytic leukemia, acute myeloid leukemia, adenocystic carcinoma, adrenocortical cancer, agnogenic myeloid metaplasia, alopecia, alveolar soft-part sarcoma, anal cancer, angiosarcoma, aplastic anemia, astrocytoma, ataxia-telangiectasia, basal cell carcinoma (skin), bladder cancer, bone cancers, bowel cancer, brain stem glioma, brain and CNS tumors, breast cancer, CNS tumors, carcinoid tumors, ...
http://www.patentsencyclopedia.com/app/20110160069
IMMUNOMODULATING COMPOSITIONS AND USES THEREFOR - Patent applicationIMMUNOMODULATING COMPOSITIONS AND USES THEREFOR - Patent application
0082]Target antigens useful in the present invention are typically proteinaceous molecules, representative examples of which include polypeptides and peptides. Such molecules may also include, for example, a non-proteinaceous moiety such as but not limited to simple intermediary metabolites, sugars, lipids, and hormones as well as macromolecules such as complex carbohydrates, phospholipids and nucleic acids. Target antigens may be selected from endogenous antigens produced by a host or exogenous antigens that are foreign to the host. Suitable endogenous antigens include, but are not restricted to, cancer or tumor antigens. Non-limiting examples of cancer or tumor antigens include antigens from a cancer or tumor selected from ABL1 protooncogene, AIDS related cancers, acoustic neuroma, acute lymphocytic leukemia, acute myeloid leukemia, adenocystic carcinoma, adrenocortical cancer, agnogenic myeloid metaplasia, alopecia, alveolar soft-part sarcoma, anal cancer, angiosarcoma, aplastic anemia, ...
http://www.patentsencyclopedia.com/app/20090136546
A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential...A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential...
Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms with variable risk of evolution into post-PV and post-ET myelofibrosis, from now on referred to as secondary myelofibrosis (SMF). No specific tools have been defined for risk stratification in SMF. To develop a prognostic model for predicting survival, we studied 685 JAK2, CALR, and MPL annotated patients with SMF. Median survival of the whole cohort was 9.3 years (95% CI: 8-not reached-NR-). Through penalized Cox regressions we identified negative predictors of survival and according to beta risk coefficients we assigned 2 points to hemoglobin level |11 g/dl, to circulating blasts ⩽3%, and to CALR-unmutated genotype, 1 point to platelet count |150 × 109/l and to constitutional symptoms, and 0.15 points to any year of age. Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM) allocated SMF patients into four risk categories with different survival (P|0.0001): low (median survival NR; 133 patients),
https://www.nature.com/articles/leu2017169.epdf?no_publisher_access=1&r3_referer=nature&error=cookies_not_supported&code=f0d3e797-b584-4f8b-87c2-bc59e1328a59
Prominent role of TGF-beta 1 in thrombopoietin-induced myelofibrosis in mice. - MRC Weatherall Institute of Molecular Medicine
Several studies suggest an implication of transforming growth factor-beta1 (TGF-beta1) in the promotion of myelofibrosis associated with hematopoietic malignancies, but the involvement of this cytokine is not fully investigated. To test directly the impact of TGF-beta1 in the pathogenesis of myelofibrosis, bone marrow stem cells from homozygous TGF-beta1 null (TGF-beta1(-/-)) and wild-type (WT) littermates were infected with a retrovirus encoding the murine thrombopoietin (TPO) protein and engrafted into lethally irradiated wild-type hosts for long-term reconstitution. Over the 4 months of follow-up, TPO levels in plasma were markedly elevated in both groups of mice, and animals typically developed a myeloproliferative syndrome characterized by thrombocytosis, leukocytosis, splenomegaly, increased numbers of progenitors in blood, and extramedullary hematopoiesis. Severe fibrosis was observed in spleen and marrow from all the mice engrafted with WT cells. In contrast, none of the mice repopulated with
https://www.imm.ox.ac.uk/publications/272258
MPN Research Foundation: TAKE ACTION NOW: MEDICARE IS ABOUT TO SET STANDARDS FOR MYELOFIBROSIS RELATED STEM CELL TRANSPLANTSMPN Research Foundation: TAKE ACTION NOW: MEDICARE IS ABOUT TO SET STANDARDS FOR MYELOFIBROSIS RELATED STEM CELL TRANSPLANTS
mpnrf advocacy (11) mpn research (10) myelofibrosis (9) essential thrombocythemia (7) mpnrf grants (5) polycythemia vera (5) patient support (4) mpn researchers (3) LLS (2) catriona jamieson (2) insurance issues (2) jak2 (2) medicare (2) mf challenge (2) myelofibrosis polycythemia vera (2) myeloproliferative neoplasms (2) patient events (2) symposia (2) treatments (2) trek for a cure (2) Benjamin Braun (1) Dorothy Sipkins (1) FDA (1) Francois Delhommeau (1) MPD brochure (1) NORD (1) Robert Kralovics (1) ayalew tefferi (1) biotech (1) chicago roundtable (1) coumadin (1) crt (1) epidemiology (1) fatigue (1) hematology (1) how to cope (1) imetelstat (1) mayo clinic (1) mf (1) momelotinib (1) mpd research alliance (1) myeloproliferative research (1) new york mpn (1) pacritinib (1) pegasys (1) prevalence (1) progression (1) pv (1) rare disease registry (1) ruben mesa (1) saghi ghaffari (1) stem cell transplant (1) support group (1) toshiaki kawakami (1) wei tong (1) ...
http://mpnrf.blogspot.com/2015/11/take-action-now-medicare-is-about-to.html
Antibiotics | Free Full-Text | Frequent Klebsiella pneumoniae Urinary Tract Infections in a Patient Treated with Ruxolitinib
Ruxolitinib is a targeted agent that inhibits Janus 2 Kinase and is approved for use in Polycythemia Vera and Primary Myelofibrosis. Its mechanism of action involves inhibition of cellular proliferation via the Janus kinase/signal transducer and activator of transcription proteins pathway. Ruxolitinib has different immune modulating effects that result in functional immunosuppression, leading to an increased susceptibility to certain infections. Klebsiella pneumoniae infections, in particular, were common among the reported pathogens contracted by ruxolitinib users. We report a 75-year-old male patient who had recurrent K. pneumoniae urinary tract infections while on ruxolitinib for Polycythemia Vera. This case is reported to add to the literature describing an increased susceptibility of patients to this often-resistant bacteria and to raise awareness about the immune modulating effects of JAK inhibitors.
https://0-www-mdpi-com.brum.beds.ac.uk/2079-6382/8/3/150
Methylome profiling reveals distinct alterations in phenotypic and mutational subgroups of myeloproliferative neoplasms<...
TY - JOUR. T1 - Methylome profiling reveals distinct alterations in phenotypic and mutational subgroups of myeloproliferative neoplasms. AU - Nischal, Sangeeta. AU - Bhattacharyya, Sanchari. AU - Christopeit, Maximilian. AU - Yu, Yiting. AU - Zhou, Li. AU - Bhagat, Tushar D.. AU - Sohal, Davendra. AU - Will, Britta. AU - Mo, Yongkai. AU - Suzuki, Masako. AU - Pardanani, Animesh. AU - Michael McDevitt, McDevitt. AU - Maciejewski, Jaroslaw P.. AU - Melnick, Ari M.. AU - Greally, John M.. AU - Steidl, Ulrich. AU - Moliterno, Alison R. AU - Verma, Amit. PY - 2013/2/1. Y1 - 2013/2/1. N2 - Even though mutations in epigenetic regulators frequently occur in myeloproliferative neoplasms, their effects on the epigenome have not been well studied. Furthermore, even though primary myelofibrosis (PMF) has a markedly worse prognosis than essential thrombocytosis or polycythemia vera, the molecular distinctions between these subgroups are not well elucidated. We conducted the HELP (HpaII tiny fragment enriched ...
https://jhu.pure.elsevier.com/en/publications/methylome-profiling-reveals-distinct-alterations-in-phenotypic-an-6
Interferon therapy in the management of myeloproliferative neoplasmsInterferon therapy in the management of myeloproliferative neoplasms
Treatment with interferon (IFN) therapy is considered successful for patients with myeloproliferative neoplasms (MPN), such as polycythemia vera (PV) and essential thrombocythemia (ET), since it has shown to deliver disease-modifying changes with durable responses and reversal of bone marrow fibrosis.
https://mpn-hub.com/medical-information/interferon-therapy-in-the-management-of-myeloproliferative-neoplasms
The spleen of patients with myelofibrosis harbors defective mesenchymal stromal cells<...
TY - JOUR. T1 - The spleen of patients with myelofibrosis harbors defective mesenchymal stromal cells. AU - Avanzini, Maria Antonietta. AU - Abbonante, Vittorio. AU - Catarsi, Paolo. AU - Dambruoso, Irene. AU - Mantelli, Melissa. AU - Poletto, Valentina. AU - Lenta, Elisa. AU - Guglielmelli, Paola. AU - Croce, Stefania. AU - Cobianchi, Lorenzo. AU - Jemos, Basilio. AU - Campanelli, Rita. AU - Bonetti, Elisa. AU - Di Buduo, Christian Andrea. AU - Salmoiraghi, Silvia. AU - Villani, Laura. AU - Massa, Margherita. AU - Boni, Marina. AU - Zappatore, Rita. AU - Iurlo, Alessandra. AU - Rambaldi, Alessandro. AU - Vannucchi, Alessandro Maria. AU - Bernasconi, Paolo. AU - Balduini, Alessandra. AU - Barosi, Giovanni. AU - Rosti, Vittorio. PY - 2018. Y1 - 2018. N2 - Splenic hematopoiesis is a major feature in the course of myelofibrosis (MF). In fact, the spleen of patients with MF contains malignant hematopoietic stem cells retaining a complete differentiation program, suggesting both a pivotal role of the ...
https://moh-it.pure.elsevier.com/en/publications/the-spleen-of-patients-with-myelofibrosis-harbors-defective-mesen
Chronic myeloproliferative disorders with myeloid metaplasia | AVESİS
Copy For Citation Kabukcuoolu S. AMERICAN JOURNAL OF SURGICAL PATHOLOGY, vol.24, no.10, pp.1437, 2000 (Journal Indexed in SCI) ...
https://avesis.ogu.edu.tr/yayin/9069aec6-8557-426d-9183-783a5851afd9/chronic-myeloproliferative-disorders-with-myeloid-metaplasia
Myeloproliferative NeoplasmsMyeloproliferative Neoplasms
Myeloproliferative neoplasms are a group of clonal myeloid cell-derived disorders characterized by myeloproliferation without dysplasia, bone marrow hypercellularity, and predisposition to thrombosis, hemorrhage, and bone marrow fibrosis.
https://www.cancernetwork.com/view/myeloproliferative-neoplasms
The New WHO classification for Essential Thrombocythemia | PV ReporterThe New WHO classification for Essential Thrombocythemia | PV Reporter
In 2016 revised classification of MPN pre-fibrotic primary myelofibrosis was recognized as a separate entity, distinct from essential thrombocythemia.
https://www.pvreporter.com/new-who-classification-for-essential-thrombocythemia-calls-for-revision-of-available-evidences/
Conditions | Richard T. Silver MD Myeloproliferative Neoplasms CenterConditions | Richard T. Silver MD Myeloproliferative Neoplasms Center
The classic chronic MPNs are polycythemia vera, essential thrombocythemia, chronic myelogenous leukemia (CML), and primary myelofibrosis. There are about 15/100,000 new cases of MPNs annually. There are more unusual MPNs, the classification of which is subject to periodic modification as we learn more about them.
https://silvermpncenter.weill.cornell.edu/patients/conditions
Lenalidomide therapy in myelofibrosis with myeloid metaplasia. - PubMed - NCBILenalidomide therapy in myelofibrosis with myeloid metaplasia. - PubMed - NCBI
Blood. 2006 Aug 15;108(4):1158-64. Epub 2006 Apr 11. Clinical Trial, Phase II; Multicenter Study; Research Support, Non-U.S. Govt
https://www.ncbi.nlm.nih.gov/pubmed/16609064
ABT-737ABT-737
The term myeloproliferative neoplasms (MPN) refers to a heterogeneous group of diseases including not only polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), but also chronic myeloid leukemia (CML), and systemic mastocytosis (SM). underlying microenvironmental changes in various MPN. Furthermore, targeting of the microenvironment in MPN is usually discussed. Such novel therapies may enhance the efficacy and may… More →. ...
http://www.ecologicalsgardens.com/tag/abt-737/
Perspectives on the impact of JAK-inhibitor therapy upon inflammation-mediated comorbidities in myelofibrosis and related...Perspectives on the impact of JAK-inhibitor therapy upon inflammation-mediated comorbidities in myelofibrosis and related...
TY - JOUR. T1 - Perspectives on the impact of JAK-inhibitor therapy upon inflammation-mediated comorbidities in myelofibrosis and related neoplasms. AU - Hasselbalch, Hans C.. PY - 2014/4. Y1 - 2014/4. N2 - Chronic inflammation is suggested to contribute to the Philadelphia- chromosome-negative myeloproliferative neoplasm (MPN) disease initiation and progression, as well as the development of premature atherosclerosis and may drive the development of other cancers in MPNs, both nonhematologic and hematologic. The MPN population has a substantial comorbidity burden, including cerebral, cardiovascular, pulmonary, abdominal, renal, metabolic, skeletal, autoimmune, and chronic inflammatory diseases. This review describes the comorbidities associated with MPNs and the potential impact of early intervention with anti-inflammatory and/or immunomodulatory agents such as JAK-inhibitors, statins, and IFN-α to inhibit cancer progression and reduce MPN-associated comorbidity impact. Early intervention may ...
https://pure-portal.regsj.dk/da/publications/perspectives-on-the-impact-of-jak-inhibitor-therapy-upon-inflamma
Myelofibrosis - Dog
Affected dogs usually present with a normocytic, normochromic nonregenerative anemia, neutropenia and thrombocytopenia. Diagnosis requires multiple bone marrow core biopsies and histological examination of other organ tissue samples, confirming the increased presence of fibrosis within bone marrow spaces and increased extramedullary hematopoiesis in other organs such as the liver and spleen. There is no specific treatment for this condition apart from long-term prednisolone, erythropoietin and addressing the underlying cause of this disease. In severely anemic dogs, whole blood transfusions of fresh frozen plasma may be required as a palliative strategy to forestall a demise. Development of new protein kinase inhibitors has shown promise at treating myelofibrosis secondary to lymphoma[13]. ...
https://www.vetbook.org/wiki/dog/index.php/Myelofibrosis
Detection of the Janus kinase 2 V617F mutation using molecular methods
In 2005, a mutation located at exon 14 of the Janus Kinase gene on chromosome 9 was discovered in patients with Polycythaenia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). The mutation (JAK2 V617F/G1849T) causes valine to be substituted by phenylalanine at codon 617. As a result the World Health Organisation (WHO) revised the diagnostic criteria of myeloproliferative neoplasms (MPN) in 2008 to include the detection of the JAK2 V617F mutation as a major diagnostic criterion for PV, ET and PMF. Molecular assays with high sensitivity and specificity should be offered by diagnostic laboratories for this purpose. To comply with these requirements, commercial and in-house assays that offer different sensitivity and specificity levels have been developed. In addition to the performance characteristics of diagnostic assays used to detect the JAK2 V617F mutation, associated cost remains an important factor to consider when selecting the assay that is best suited to a ...
https://repository.up.ac.za/handle/2263/53045
8p11 myeloproliferative syndrome: diagnostic challenges and pitfalls. | PubFacts8p11 myeloproliferative syndrome: diagnostic challenges and pitfalls. | PubFacts
8p11 myeloproliferative syndrome (EMS) is a very rare clinicopathological entity which is characterized by the appearance of a myeloproliferative neoplasm in the bone marrow, peripheral lymphadenopathy, usually caused by T or B lymphoblastic lymphoma/leukemia, and a reciprocal translocation involving chromosome 8p11. Herein we describe a 22-year-old male patient with unusual clinical presentation of EMS. Namely, he initially presented with prolonged epistaxis. Complete blood count showed elevated hemoglobin (17.7g/dl), thrombocytopenia (98x109/l) and leukocytosis (57x109/l). Bone marrow aspirate and biopsy findings corresponded with the presence of a myeloproliferative neoplasm while cytogenetic analysis revealed t(8;13)(p11q12). After that ZMYM2-FGFR1 in-frame fusion was confirmed at the molecular level. Immediately after establishing the diagnosis of a myeloproliferative neoplasm (MPN) generalized lymphadenopathy was developed. Histopathologic examination of lymph node sample confirmed the ...
https://www.pubfacts.com/detail/27569099/8p11-myeloproliferative-syndrome-diagnostic-challenges-and-pitfalls
Carcinosinum (58T)Carcinosinum (58T)
This is not yet a true case study but for people following my posts on my website, I wanted to write about the great healing potential this remedy, Carcinosinum (58T) seems to have.. The basic Carcinosinum that we use is a remedy prepared from a single tumour - a breast tumour. This is the remedy of which Foubister made a detailed proving. Tinus Smits from Netherlands has used Carcinosinum (15T) with good results, better than those got from our regular single tumour Carcinosinum.. On a forum post at hpathy.com, there was a detailed discussion on the use of Carcinosinum (58T) http://forum.hpathy.com/forum/students-corner/carcinosinum/#p8599. Having my interest triggered, I ordered Carcinosinum (58T) in two potencies, 200c and 1M from Remedia Homoeopathic Pharmacy, in Austria.. I got the chance to use it after a few months of procuring it in a case of thrombocytosis and bone marrow fibrosis (that was secondary to the chemotherapeutic treatment for the thrombocytosis).. His allopathic treatment ...
http://www.homoeopathie.in/2015/06/02/carcinosinum-58t/
Risk Factors in Myelofibrosis Linked With Poor Outcome After Splenectomy | Cancer Network | The Oncology JournalRisk Factors in Myelofibrosis Linked With Poor Outcome After Splenectomy | Cancer Network | The Oncology Journal
Survival of patients with myelofibrosis who undergo splenectomy is adversely affected by older age, the need for transfusion, and leukocyte and circulating blast cell counts, according to a new analysis. 1
http://www.cancernetwork.com/hematologic-malignancies/risk-factors-myelofibrosis-linked-poor-outcome-after-splenectomy
SAT0480 Bone Marrow Lesion in Advanced Osteoarthritis of The Knees. Correlation Study between Histopathological Findings and...SAT0480 Bone Marrow Lesion in Advanced Osteoarthritis of The Knees. Correlation Study between Histopathological Findings and...
Results The histopathology of BML in cases of OA revealed that 6 biopsies of cases showing bone marrow fibrosis (30%), 4 of them grade 1 (20%) and 2 of them grade 2 (10%). 18 biopsies showing cyst (90%), 9 biopsies showing abnormal trabeculae (45%), 2 of them with grade 1 (10%), 4 of them grade 2 (20%) and 3 of them grade 3 (15%). 5 biopsies showing lymphocyte (25%), 40% of them had ++CD3, while 60% of them had ++CD20. 5 biopsies showing fatty marrow (25%), 9 biopsies showing haemosidrotic marrow (45%), 6 biopsies showing blood vessels (30%), 5 of them with grade 2 (25%) and 1 with grade 3 (5%).. The MRI findings of OA patients had been revealed that there were 6 patients with BML of grade 1 (30%), 10 patients of grade 2 (50%) and 4 patients of grade 3 (20%). ...
http://ard.bmj.com/content/75/Suppl_2/844.1
Myelofibrosis Awareness DayMyelofibrosis Awareness Day
CancerCare and the advocacy associations that comprise the MPN Coalition, recognizes the first ever national Myelofibrosis Awareness Day.
https://www.cancercare.org/press/releases/77-2012_09_20
Myelofibrosis Diagnosis: Costs for treatment #225091 in Germany | BookingHealthMyelofibrosis Diagnosis: Costs for treatment #225091 in Germany | BookingHealth
Myelofibrosis Diagnosis (costs for program #225091) ✔ University Hospital Würzburg ✔ Department of Pediatric and Adolescent Medicine ✔ BookingHealth.com
https://bookinghealth.com/university-hospital-of-w-rzburg/diagnostic-programs/225091-myelofibrosis-diagnosis.html
Myelofibrosis - Market Insights, Epidemiology and Market Forecast - 2025 - RnR Market ResearchMyelofibrosis - Market Insights, Epidemiology and Market Forecast - 2025 - RnR Market Research
Myelofibrosis - Market Insights, Epidemiology and Market Forecast - 2025 is a market research report available at US $5750 for a Single User PDF License from RnR Market Research Reports Library.
http://www.rnrmarketresearch.com/myelofibrosis-market-insights-epidemiology-and-market-forecast-2025-market-report.html
Myelofibrosis Drug Granted Orphan Drug Status - MPRMyelofibrosis Drug Granted Orphan Drug Status - MPR
The Food and Drug Admnistration (FDA) has granted Orphan Drug designation for PRM-151 (Promedior) for the treatment of myelofibrosis.
http://www.empr.com/drugs-in-the-pipeline/myelofibrosis-drug-granted-orphan-drug-status/article/369317/
Items where Author is Prka, Željko - Repozitorij Medicinskog fakulteta Sveučilišta u ZagrebuItems where Author is Prka, Željko - Repozitorij Medicinskog fakulteta Sveučilišta u Zagrebu
Lucijanić, Marko and Livun, Ana and Tomasović-Lončarić, Čedna and Štoos-Veić, Tajana and Pejša, Vlatko and Jakšić, Ozren and Prka, Željko and Kušec, Rajko (2016) Canonical Wnt/β-catenin signaling pathway is dysregulated in patients with primary and secondary myelofibrosis. Clinical Lymphoma Myeloma and Leukemia, 16 (9). pp. 523-6. ISSN 2152-2650 Lucijanić, Marko and Pejša, Vlatko and Mitrović, Zdravko and Štoos-Veić, Tajana and Livun, Ana and Jakšić, Ozren and Vasilj, Tamara and Piršić, Mario and Hariš, Višnja and Prka, Željko and Kušec, Rajko (2016) Hemochromatosis gene mutations may affect the survival of patients with myelodysplastic syndrome. Hematology, 21 (3). pp. 170-4. ISSN 1024-5332 Lucijanić, Marko and Pejša, Vlatko and Jakšić, Ozren and Mitrović, Zdravko and Tomasović-Lončarić, Čedna and Štoos-Veić, Tajana and Prka, Željko and Piršić, Mario and Hariš, Višnja and Vasilj, Tamara and Kušec, Rajko (2016) The degree of anisocytosis predicts ...
http://medlib.mef.hr/view/creators/Prka=3A==017Deljko_=3A=3A.default.html
Items where Author is Pejša, Vlatko - Repozitorij Medicinskog fakulteta Sveučilišta u ZagrebuItems where Author is Pejša, Vlatko - Repozitorij Medicinskog fakulteta Sveučilišta u Zagrebu
Lucijanić, Marko and Livun, Ana and Tomasović-Lončarić, Čedna and Štoos-Veić, Tajana and Pejša, Vlatko and Jakšić, Ozren and Prka, Željko and Kušec, Rajko (2016) Canonical Wnt/β-catenin signaling pathway is dysregulated in patients with primary and secondary myelofibrosis. Clinical Lymphoma Myeloma and Leukemia, 16 (9). pp. 523-6. ISSN 2152-2650 Lucijanić, Marko and Pejša, Vlatko and Mitrović, Zdravko and Štoos-Veić, Tajana and Livun, Ana and Jakšić, Ozren and Vasilj, Tamara and Piršić, Mario and Hariš, Višnja and Prka, Željko and Kušec, Rajko (2016) Hemochromatosis gene mutations may affect the survival of patients with myelodysplastic syndrome. Hematology, 21 (3). pp. 170-4. ISSN 1024-5332 Lucijanić, Marko and Pejša, Vlatko and Jakšić, Ozren and Mitrović, Zdravko and Tomasović-Lončarić, Čedna and Štoos-Veić, Tajana and Prka, Željko and Piršić, Mario and Hariš, Višnja and Vasilj, Tamara and Kušec, Rajko (2016) The degree of anisocytosis predicts ...
http://medlib.mef.hr/view/creators/Pej==0161a=3AVlatko=3A=3A.html
Dry Tap Bone Marrow & Pallor: Causes & Reasons - SymptomaDry Tap Bone Marrow & Pallor: Causes & Reasons - Symptoma
Dry Tap Bone Marrow & Pallor Symptom Checker: Possible causes include Primary Myelofibrosis. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.
https://www.symptoma.com/en/ddx/dry-tap-bone-marrow+pallor
8p11 myeloproliferative syndrome: a review8p11 myeloproliferative syndrome: a review
The 8p11 myeloproliferative syndrome is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 tyrosine kinase gene on chromosome 8p11-12. By our count, 65 cases are currently reported in the literature. This neoplasm affects patients of a …
https://pubmed.ncbi.nlm.nih.gov/20226962/?dopt=Abstract
CHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME | MENDELIAN.COCHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME | MENDELIAN.CO
CHROMOSOME 8p11 MYELOPROLIFERATIVE SYNDROME description, symptoms and related genes. Get the complete information in our medical search engine for phe
https://www.mendelian.co/diseases/chromosome-8p11-myeloproliferative-syndrome
PD-1 antibody and ruxolitinib enhances graft-versus-lymphoma effect without increasing acute graft-versus-host disease in mice
cells. Given alone the PD-1 antibody increased GVL but did not improve survival of recipients challenged with A20 cells because of increased deaths from aGVHD. Adding ruxolitinib decreased levels of effector T cells and related cytokines. Tbx21- T cells had higher PD-1 levels compared with Tbx21+ T cells. Ruxolitinib increased PD-1 levels on donor T cells by suppressing Tbx21 expression. Ruxolitinib increased apoptosis of T cells which was reversed by the PD-1 antibody. PD-1 antibody preserved expression of granzyme B and cytotoxicity of T cells which were decreased by ruxolitinib. The net result of combined therapy was increased GVL, no increase in aGVHD and increased survival. The combined therapy improved survival of recipients challenged by A20 cells which expressed high level of PD-L1, but not EL4 cells which do not express PD-L1. ...
https://health.omgcb.com/lymphoma/6977/pd-1-antibody-and-ruxolitinib-enhances-graft-versus-lymphoma-effect-without-increasing-acute-graft-versus-host-disease-in-mice.html
An MPN Story: Planning for the Future 14 Years After Diagnosis | Myeloproliferative Neoplasms | Patient PowerAn MPN Story: Planning for the Future 14 Years After Diagnosis | Myeloproliferative Neoplasms | Patient Power
Margie Lunt is planning for her future in spite of living with a myeloproliferative neoplasm (MPN), known as myelofibrosis (MF). Hear how Margies faith and positive outlook allow her to live a full life with MF.
https://patientpower.info/video/an-mpn-story-planning-for-the-future-years-after-diagnosis
Differential association of calreticulin type 1 and type 2 mutations with myelofibrosis and essential thrombocytemia: relevance...Differential association of calreticulin type 1 and type 2 mutations with myelofibrosis and essential thrombocytemia: relevance...
Nangalia J., Massie C.E., Baxter E.J., Nice F.L., Gundem G., Wedge D.C., Avezov E., Li J., Kollmann K., Kent D.G., Aziz A., Godfrey A.L., Hinton J., Martincorena I., Van Loo P., Jones A.V., Guglielmelli P., Tarpey P., Harding H.P., Fitzpatrick J.D., Goudie C.T., Ortmann C.A., Loughran S.J., Raine K., Jones D.R., Butler A.P., Teague J.W., OMeara S., McLaren S., Bianchi M., Silber Y., Dimitropoulou D., Bloxham D., Mudie L., Maddison M., Robinson B., Keohane C., Maclean C., Hill K., Orchard K., Tauro S., Du M.-Q., Greaves M., Bowen D., Huntly B.J.P., Harrison C.N., Cross N.C.P., Ron D., Vannucchi A.M., Papaemmanuil E., Campbell P.J., Green A.R., Somatic CALR Mutations in Myeloproliferative Neoplasms with Nonmutated JAK2, 10.1056/nejmoa1312542 ...
https://dial.uclouvain.be/pr/boreal/object/boreal:155936
Download Cardiovascular System, Red Blood Cells, And Oxygen Transport In Microgravity
for apply receive funds deposited and done. Hard to most comfortable rates will first of the earliest cookies, not those shedding experimentally to the results and long, are much not main and only modern. We m dating these superluminal problems in online, 2nd download, literary inequalities, emitting the other book and cardiography. A similar and English simple download Cardiovascular System, Red s the once other byVictor and of her dynamic international airflow. providing five descriptions of biographical download Cardiovascular System, Red Blood Cells, to this straight order, she ich the ministry about range, outlet, implementation, and herself, enlarged with spoilers of all the low philosophers who were her model for a embarrassed community. download Cardiovascular System, Red web programs are going, flying, and shedding the environment and bookmark of little career with share, being comments along with a beauty of afterburner simulations and transport intervals to provide major, intrinsic ...
http://michaeltiemann.com/docs/ebooks/download-Cardiovascular-System%2C-Red-Blood-Cells%2C-and-Oxygen-Transport-in-Microgravity.php
Celgene to Acquire Impact Biomedicines, Adding Fedratinib to Its Pipeline of Novel Therapies for Hematologic Malignancies ...Celgene to Acquire Impact Biomedicines, Adding Fedratinib to Its Pipeline of Novel Therapies for Hematologic Malignancies ...
Fedratinib is a highly selective JAK2 kinase inhibitor that is being evaluated for myelofibrosis and polycythemia vera Fedratinib demonstrated clinical improvement in a phase III trial with treatment-naïve myelofibrosis patie...
http://ir.celgene.com/releasedetail.cfm?releaseid=1053509
Primary myelofibrosisPrimary myelofibrosis
... the primary diagnostic difference being the grade of fibrosis. The primary feature of primary myelofibrosis is bone marrow ... Primary Myelofibrosis, Merck. Cervantes F (March 2005). "Modern management of myelofibrosis". British Journal of Haematology. ... Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a ... Primary myelofibrosis can begin with a blood picture similar to that found in polycythemia vera or chronic myeloid leukemia. ...
https://en.wikipedia.org/wiki/Primary_myelofibrosis
Prefibrotic primary myelofibrosisPrefibrotic primary myelofibrosis
"Evidence that Prefibrotic Myelofibrosis Is Aligned along a Clinical and Biological Continuum Featuring Primary Myelofibrosis". ... Prefibrotic primary myelofibrosis (Pre-PMF) is a rare blood cancer, classified by the World Health Organization as a distinct ... The disease is progressive to overt primary myelofibrosis, though the rate of progression is variable and not all patients ... Reticulin or collagen fibrosis grade 2 or 3 is a diagnostic criteria for primary myelofibrosis. Both pre-PMF and Essential ...
https://en.wikipedia.org/wiki/Prefibrotic_primary_myelofibrosis
Mir-190 microRNA precursor familyMir-190 microRNA precursor family
"MicroRNA expression profile in granulocytes from primary myelofibrosis patients". Experimental Hematology. 35 (11): 1708-18. ...
https://en.wikipedia.org/wiki/Mir-190_microRNA_precursor_family
GATA1GATA1
"Downregulation of GATA1 drives impaired hematopoiesis in primary myelofibrosis". The Journal of Clinical Investigation. 127 (4 ... Myelofibrosis is a rare hematological malignancy characterized by progressive fibrosis of the bone marrow, extramedullary ... Reduced levels of GATA1 due to defective translation of GATA1 mRNA in human megakaryocytes is associated with myelofibrosis, i. ... Based primarily on mouse and isolated human cell studies, this myelofibrosis is thought to result from the accumulation of ...
https://en.wikipedia.org/wiki/GATA1
Transient myeloproliferative diseaseTransient myeloproliferative disease
"Downregulation of GATA1 drives impaired hematopoiesis in primary myelofibrosis". The Journal of Clinical Investigation. 127 (4 ... The liver, it is suggested, may be the primary site for excessive proliferation of the GATA1 mutant clone(s) of platelet ... Based primarily on mouse and isolated human cell studies, this myelofibrosis is thought to result from the excessive ... "CXCR4-independent rescue of the myeloproliferative defect of the Gata1low myelofibrosis mouse model by Aplidin". Journal of ...
https://en.wikipedia.org/wiki/Transient_myeloproliferative_disease
Acute megakaryoblastic leukemiaAcute megakaryoblastic leukemia
... and primary myelofibrosis. In one review of adult-AMKL, 25% of 49 cases were considered as secondary to one of these MPN. The ... primary myelofibrosis, or mediastinal germ cell tumor. AMKL associated with mediastinal germ cell tumors typically occurs in ... has many clinical and laboratory features similar to and must be distinguished from Acute panmyelosis with myelofibrosis, a ...
https://en.wikipedia.org/wiki/Acute_megakaryoblastic_leukemia
CD146CD146
January 2009). "CD146(+) bone marrow osteoprogenitors increase in the advanced stages of primary myelofibrosis". Haematologica ...
https://en.wikipedia.org/wiki/CD146
ThrombocythemiaThrombocythemia
These include: essential thrombocythemia, chronic myelogenous leukemia, polycythemia vera, and primary myelofibrosis. Extremely ... as either primary thrombocythemia or essential thrombocythemia. The condition arises from a fault in the bone marrow cells ... especially primary thrombocytosis) is a potential cause of thrombophilia. Conversely, secondary thrombocytosis very rarely ...
https://en.wikipedia.org/wiki/Thrombocythemia
Myeloproliferative neoplasmMyeloproliferative neoplasm
Prefibrotic/early primary myelofibrosis Prefibrotic primary myelofibrosis (Pre-PMF) is typically associated with JAK2, CALR, or ... Overtly fibrotic myelofibrosis Like pre-PMF, overt primary myelofibrosis is associated with JAK2, CALR, or MPL mutations. ... In rare cases, some MPNs such as primary myelofibrosis may accelerate and turn into acute myeloid leukemia. MPNs are classified ... Recently, a JAK2 inhibitor, namely ruxolitinib, has been approved for use in primary myelofibrosis. Trials of these inhibitors ...
https://en.wikipedia.org/wiki/Myeloproliferative_neoplasm
JADE1JADE1
The study identified seven novel deletions and translocations in small cohort of patients with primary myelofibrosis. JADE1 and ... A recent study searched for novel submicroscopic genetic changes in myelofibrosis, which is a bone marrow cancer. ... "Identification of submicroscopic genetic changes and precise breakpoint mapping in myelofibrosis using high resolution mate- ...
https://en.wikipedia.org/wiki/JADE1
Severe congenital neutropeniaSevere congenital neutropenia
June 2013). "The Thr224Asn mutation in the VPS45 gene is associated with the congenital neutropenia and primary myelofibrosis ... A subset of SCN4 has severe primary pulmonary hypertension and respiratory failure. SCN5 arises from autosomal recessive ... Unlike classical Kostmann disease, SCN5 also has defective platelet aggregation (thrombasthenia) and myelofibrosis. This type ... although this may increase risk for myelofibrosis and acute myeloid leukemia in the long term. Over 90% of SCN responds to ...
https://en.wikipedia.org/wiki/Severe_congenital_neutropenia
Immunomodulatory imide drugImmunomodulatory imide drug
EMA has already granted pomalidomide an orphan designation for primary myelofibrosis, MM, systemic sclerosis, post- ... amyloidosis Primary myelofibrosis (PMF) Acute myeloid leukaemia (AML) Prostate cancer Metastatic renal cell carcinoma (mRCC) ... The primary use of IMiDs in medicine is in the treatment of cancers and autoimmune diseases (including one that is a response ... Described below are schemes for synthesizing thalidomide, lenalidomide, and pomalidomide, as reported from prominent primary ...
https://en.wikipedia.org/wiki/Immunomodulatory_imide_drug
Janus kinase inhibitorJanus kinase inhibitor
"Momelotinib in Transfusion-Dependent Adults with Primary Myelofibrosis (PMF) or Post-polycythemia Vera or Post-essential ... "U.S. FDA Approves INREBIC® (Fedratinib) as First New Treatment in Nearly a Decade for Patients With Myelofibrosis". ir.celgene. ... Vaddi K, Sarlis NJ, Gupta V (November 2012). "Ruxolitinib, an oral JAK1 and JAK2 inhibitor, in myelofibrosis". Expert Opinion ... "Pacritinib in Combination with Low Dose Decitabine in Intermediate-High Risk Myelofibrosis or Myeloproliferative Neoplasm (MPN ...
https://en.wikipedia.org/wiki/Janus_kinase_inhibitor
MomelotinibMomelotinib
As of 2016, momelotinib is being investigated for primary myelofibrosis or post-polycythemia vera or post-essential ... "Momelotinib in Transfusion-Dependent Adults with Primary Myelofibrosis (PMF) or Post-polycythemia Vera or Post-essential ... momelotinib is being developed as a drug for myelofibrosis and currently undergoes Phase I/II clinical trials. Additional ... in vitro Assessment of Kinase Selectivity and Preclinical Studies Using Cell Lines and Primary Cells from Polycythemia vera ...
https://en.wikipedia.org/wiki/Momelotinib
Chronic myelogenous leukemiaChronic myelogenous leukemia
... and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092-7. doi:10.1182/ ...
https://en.wikipedia.org/wiki/Chronic_myelogenous_leukemia
Alkaline phosphataseAlkaline phosphatase
... and in primary myelofibrosis. Lower than typical levels are found in pathologies that involve undeveloped leukocytes, such as ... "Association between serum alkaline phosphatase and primary resistance to erythropoiesis stimulating agents in chronic kidney ... Bone conditions Osteoblastic bone tumors Osteomalacia Osteoporosis Hepatitis Cirrhosis Acute cholecystitis Myelofibrosis ...
https://en.wikipedia.org/wiki/Alkaline_phosphatase
Virtual karyotypeVirtual karyotype
... and primary myelofibrosis show an inherent tendency for transformation into leukemia (MPN-blast phase), which is accompanied by ... SNP array karyotyping can be used to distinguish, for example, a medulloblastoma with an isochromosome 17q from a primary ... primary tumor specimens by use of affymetrix single-nucleotide-polymorphism genotyping microarrays". Am J Hum Genet. 81 (1): ...
https://en.wikipedia.org/wiki/Virtual_karyotype
PMFPMF
... a US government fellowship Primary myelofibrosis, a disease affecting the bone marrow. Probability mass function, in statistics ...
https://en.wikipedia.org/wiki/PMF
Tumors of the hematopoietic and lymphoid tissuesTumors of the hematopoietic and lymphoid tissues
BCR-ABL1-positive Chronic neutrophilic leukaemia Polycythamemia vera Primary myelofibrosis Essential thrombocythemia Chronic ... Lymphomatoid papulosis Primary cutaneous anaplastic large cell lymphoma Primary cutaneous gamma delta T-cell lymphoma Primary ... Table 1.4: Age-Adjusted SEER Incidence and U.S. Death Rates and 5-Year Relative Survival Rates By Primary Cancer Site, Sex and ... centre B-cell subtype Activated B-cell subtype T-cell/histiocyte-rich large B-cell lymphoma Primary DLBCL of the CNS Primary ...
https://en.wikipedia.org/wiki/Tumors_of_the_hematopoietic_and_lymphoid_tissues
EosinophiliaEosinophilia
... primary myelofibrosis, chronic neutrophilic leukemia, chronic myelomonocytic leukemia, atypical chronic myelogenous leukemia, ... In primary cutaneous T cell lymphoma, blood and dermal eosinophilia are often seen. Lymphoma cells have also been shown to ... However, in primary eosinophilia, or if the eosinophil count must be lowered, corticosteroids such as prednisone may be used. ... Primary immunodeficiency diseases are inborn errors in the immune system due to defective genes. Certain of these disorders are ...
https://en.wikipedia.org/wiki/Eosinophilia
RuxolitinibRuxolitinib
... ruxolitinib is indicated for the treatment of disease-related splenomegaly or symptoms in adults with primary myelofibrosis ( ... post-polycythaemia-vera myelofibrosis, or post-essential thrombocythaemia myelofibrosis. It is also indicated for the treatment ... In myelofibrosis, the most common side effects include thrombocytopenia (low blood platelet counts), anaemia (low red blood ... In March 2012 the phase III Controlled Myelofibrosis Study with Oral JAK Inhibitor-I (COMFORT-I) and COMFORT-II trials showed ...
https://en.wikipedia.org/wiki/Ruxolitinib
Essential thrombocythemiaEssential thrombocythemia
... mutations in a majority of JAK2-negative/MPL-negative patients with essential thrombocythemia and primary myelofibrosis, which ... No evidence of myelofibrosis no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0-4 scale) B6. No evidence of a ... It may, albeit rarely, develop into acute myeloid leukemia or myelofibrosis. It is a type of myeloproliferative neoplasm (blood ... Branehog I, Ridell B, Swolin B, Weinfeld A (1975). "Megakaryocyte quantifications in relation to thrombokinetics in primary ...
https://en.wikipedia.org/wiki/Essential_thrombocythemia
PacritinibPacritinib
... adults who have a rare form of a bone marrow disorder known as intermediate or high-risk primary or secondary myelofibrosis and ... Pacritinib, sold under the brand name Vonjo, is an anti-cancer medication used to treat myelofibrosis. It is a macrocyclic ... were demonstrated in a study that included 63 participants with intermediate or high-risk primary or secondary myelofibrosis ...
https://en.wikipedia.org/wiki/Pacritinib
Index of oncology articlesIndex of oncology articles
... primary central nervous system lymphoma - primary endpoint - primary myelofibrosis - primary peritoneal cancer - primary tumor ... cancer of unknown primary origin - cancer stem cell - cancer vaccine - Cancer.gov - Candidiasis - Candidosis - CAP-1 - ... idiopathic myelofibrosis - idoxifene - idoxuridine - ifosfamide - IH636 grape seed extract - IL-1 - IL-1-alfa - IL-11 - IL-12 ... second primary cancer - second-line therapy - second-look surgery - secondary cancer - sedoxantrone trihydrochloride - ...
https://en.wikipedia.org/wiki/Index_of_oncology_articles
Elevated alkaline phosphataseElevated alkaline phosphatase
Infectious mononucleosis Primary sclerosing cholangitis Polycythemia vera Myelofibrosis Mastocytosis Leukemoid reaction to ... Liver (liver ALP): Cholestasis, cholecystitis, cholangitis, cirrhosis, primary biliary cholangitis, primary sclerosis ... Other bone metastases Renal osteodystrophy Fractured bone Skeletal involvement of other primary diseases: Osteomalacia, rickets ...
https://en.wikipedia.org/wiki/Elevated_alkaline_phosphatase
DacrocyteDacrocyte
These tear drop cells are found primarily in diseases with bone marrow fibrosis, such as: primary myelofibrosis, ... Rare causes are myelofibrosis associated with post-irradiation, toxins, autoimmune diseases, metabolic conditions, inborn ... As dacrocytes are associated with myelofibrosis, they are also theorized to be formed due to mechanically squeezing out from ...
https://en.wikipedia.org/wiki/Dacrocyte
Basophilic stipplingBasophilic stippling
... deficiency Alcoholism Myelodysplastic syndromes Sideroblastic anemia Congenital dyserythropoietic anemia Primary myelofibrosis ...
https://en.wikipedia.org/wiki/Basophilic_stippling
FedratinibFedratinib
... fedratinib is indicated for the treatment of disease-related splenomegaly or symptoms in adults with primary myelofibrosis, ... or intermediate-risk primary or post-polycythemia vera/essential thrombocythemia myelofibrosis have been published in 2011. In ... Patients with myelofibrosis frequently harbor mutations which activate the JAK-STAT signaling pathway and which are sensitive ... Myelofibrosis is a myeloid cancer associated with anemia, splenomegaly, and constitutional symptoms. ...
https://en.wikipedia.org/wiki/Fedratinib
CalreticulinCalreticulin
... mutations in a majority of JAK2-negative/MPL-negative patients with essential thrombocythemia and primary myelofibrosis, which ...
https://en.wikipedia.org/wiki/Calreticulin
OsteopetrosisOsteopetrosis
... myelofibrosis (primary disorder or secondary to intoxication or malignancy), Erdheim-Chester disease, osteosclerosing types of ...
https://en.wikipedia.org/wiki/Osteopetrosis
List of ICD-9 codes 280-289: diseases of the blood and blood-forming organsList of ICD-9 codes 280-289: diseases of the blood and blood-forming organs
... blood and blood-forming organs 289.81 Primary hypercoagulable state 289.82 Secondary hypercoagulable state 289.83 Myelofibrosis ... primary 287.31 Immune thrombocytopenic purpura Idiopathic thrombocytopenic purpura 287.4 Thrombocytopenia, secondary 287.9 ...
https://en.wikipedia.org/wiki/List_of_ICD-9_codes_280–289:_diseases_of_the_blood_and_blood-forming_organs
Idiopathic multicentric Castleman diseaseIdiopathic multicentric Castleman disease
iMCD patients with thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly syndrome (TAFRO syndrome) are ... and primary lymph node plasmacytoma. Due to the rarity of iMCD, data regarding treatment is limited and based on a combination ... myelofibrosis, renal dysfunction, and organomegaly syndrome (TAFRO syndrome). Diagnosis of iMCD requires: the presence of both ...
https://en.wikipedia.org/wiki/Idiopathic_multicentric_Castleman_disease
List of skin conditionsList of skin conditions
... folliculitis Primary cutaneous aspergillosis Primary cutaneous coccidioidomycosis Primary cutaneous histoplasmosis Primary ... Cutaneous myelofibrosis Cutaneous myxoma Cutis marmorata telangiectatica congenita (congenital generalized phlebectasia, Van ... primary neuroendocrine carcinoma of the skin, primary small cell carcinoma of the skin, trabecular carcinoma of the skin) ... Primary cutaneous immunocytoma Primary cutaneous marginal zone lymphoma Retiform parapsoriasis Secondary cutaneous CD30+ large ...
https://en.wikipedia.org/wiki/List_of_skin_conditions
William VainchenkerWilliam Vainchenker
Calreticulin mutants in mice induce an MPL-dependent thrombocytosis with frequent progression to myelofibrosis », Blood, 2016 ... F mutation triggers erythropoietin hypersensitivity and terminal erythroid amplification in primary cells from patients with ...
https://en.wikipedia.org/wiki/William_Vainchenker
List of OMIM disorder codesList of OMIM disorder codes
... primary open angle, adult onset; 137760; GLC1B Glaucoma 3, primary congenital, D; 613086; LTBP2 Glaucoma 3A, primary congenital ... STAT1 Myelofibrosis, idiopathic; 254450; JAK2 Myeloperoxidase deficiency; 254600; MPO Myeloproliferative disorder with ... primary, type 1; 259900; AGXT Hyperoxaluria, primary, type II; 260000; GRHPR Hyperoxaluria, primary, type III; 613616; DHDPSL ... primary, 12; 612650; RSPH9 Ciliary dyskinesia, primary, 13; 613193; LRRC50 Ciliary dyskinesia, primary, 3, with or without ...
https://en.wikipedia.org/wiki/List_of_OMIM_disorder_codes
Margaret McFarlandMargaret McFarland
She did not publish much academic literature; her primary impacts came from her direct work with families at the Arsenal Center ... McFarland was diagnosed with a bone marrow disorder called myelofibrosis in the 1970s, and by 1987 she was receiving blood ... In 1966, Rogers began working on Mister Rogers' Neighborhood, and McFarland became the primary consultant to the show. She ... and McFarland and Rogers continued to meet until her death from myelofibrosis at the age of 83. McFarland, the youngest of ...
https://en.wikipedia.org/wiki/Margaret_McFarland
LeukopeniaLeukopenia
It has been associated with chemotherapy, radiation therapy, myelofibrosis, aplastic anemia (failure of white cell, red cell ... and are the body's primary defense against an infection. Thus the condition of leukopenia places individuals at increased risk ...
https://en.wikipedia.org/wiki/Leukopenia
Polycythemia veraPolycythemia vera
In primary polycythemia, there may be 8 to 9 million and occasionally 11 million erythrocytes per cubic millimeter of blood (a ... myelofibrosis and leukemia". Haematologica. 88 (1): 13-8. PMID 12551821. Anía B, Suman V, Sobell J, Codd M, Silverstein M, ... or myelofibrosis. The condition is considered chronic; no cure exists. Symptomatic treatment (see below) can normalize the ... being a primary polycythemia, is caused by neoplastic proliferation and maturation of erythroid, megakaryocytic and ...
https://en.wikipedia.org/wiki/Polycythemia_vera
List of diseases (M)List of diseases (M)
... idiopathic Myelofibrosis Myelofibrosis-osteosclerosis Myeloid splenomegaly Myeloperoxidase deficiency Myhre-Ruvalcaba-Graham ... primary autosomal recessive Microco Microcoria, congenital Microcornea correctopia macular hypoplasia Microcornea glaucoma ... Mycositis fungoides Myelinopathy Myelitis Myelocerebellar disorder Myelodysplasia Myelodysplastic syndromes Myelofibrosis, ...
https://en.wikipedia.org/wiki/List_of_diseases_(M)
PancytopeniaPancytopenia
"Function and Malfunction of Hematopoietic Stem Cells in Primary Bone Marrow Failure Syndromes". Current Stem Cell Research & ... Aplastic anemia Gaucher's disease Metastatic carcinoma of bone Multiple Myeloma Overwhelming infections Lymphoma Myelofibrosis ...
https://en.wikipedia.org/wiki/Pancytopenia
LestaurtinibLestaurtinib
Phase I results were reported in 2015 for a lestaurtinib trial involving patients with V617F JAK2 positive myelofibrosis. ... June 2008). "Lestaurtinib (CEP701) is a JAK2 inhibitor that suppresses JAK2/STAT5 signaling and the proliferation of primary ... 2015). "Phase I dose escalation study of lestaurtinib in patients with myelofibrosis". Leukemia & Lymphoma. 56 (9): 2543-51. ... with the primary adverse event reported being gastrointestinal reaction. A Phase II study in 18 patients with pancreatic cancer ...
https://en.wikipedia.org/wiki/Lestaurtinib
Bone marrowBone marrow
In birds and mammals, bone marrow is the primary site of new blood cell production (or haematopoiesis). It is composed of ... marrow T1 hypointensity without contrast enhancement or cortical discontinuity suggests red marrow conversion or myelofibrosis ... The bone marrow and thymus constitute the primary lymphoid tissues involved in the production and early selection of ... which can be isolated from the primary culture of bone marrow stroma, can give rise to bone, adipose, and cartilage tissue. The ...
https://en.wikipedia.org/wiki/Bone_marrow
Thrombopoietin receptorThrombopoietin receptor
In myelofibrosis, a mutation occurs at position 515. These mutations lead to the production of thrombopoietin receptors that ... in primary cells and mouse models of myeloproliferative neoplasms". Blood Cancer J. 1 (7): e29. doi:10.1038/bcj.2011.29. PMC ... Specific mutations to this gene are associated with myelofibrosis and essential thrombocythemia. In essential thrombocythemia, ... and insulin receptor substrate proteins in BAF3 cells and primary murine megakaryocytes". J. Biol. Chem. 276 (4): 2494-502. doi ...
https://en.wikipedia.org/wiki/Thrombopoietin_receptor
Janus kinase 2Janus kinase 2
An inhibitor of JAK2-STAT5, AZD1480, was pointed as having activity in primary and CRPC. Jak2 mutation, when demonstrable, is ... Mutations in JAK2 have been implicated in polycythemia vera, essential thrombocythemia, and myelofibrosis as well as other ... "Pharmacologic inhibition of Jak2-Stat5 signaling By Jak2 inhibitor AZD1480 potently suppresses growth of both primary and ...
https://en.wikipedia.org/wiki/Janus_kinase_2
BasophiliaBasophilia
... on its own does not cause much complication other than those related to the primary causative condition. However, ... myelofibrosis, thrombocythemia, or, in rare cases, solid tumors. Alternative root causes other than these neoplasmic ...
https://en.wikipedia.org/wiki/Basophilia
Andrew LatimerAndrew Latimer
... cite Latimer as one of their primary influences. Musician and producer Steven Wilson of Porcupine Tree is a known fan of Camel ... which had unexpectedly progressed to myelofibrosis. In November 2007, he underwent a successful bone marrow transplant and ...
https://en.wikipedia.org/wiki/Andrew_Latimer
Nuclear labor issuesNuclear labor issues
The study evaluated 31 types of cancers, primary and secondary. In 1942 thirty indigenous Dené men were recruited to mine ... myelofibrosis and cancers. During the nuclear weapons testing in the Marshall Islands approximately 300,000 GIs were exposed to ...
https://en.wikipedia.org/wiki/Nuclear_labor_issues
Chronic liver diseaseChronic liver disease
... previously known as primary biliary cirrhosis) Primary sclerosing cholangitis Autoimmune Hepatitis Other Right heart failure ... myelofibrosis and metabolic abnormalities such as Gaucher's disease and glycogen storage diseases.[citation needed] Portal ... Metabolic Non-alcoholic fatty liver disease Haemochromatosis Wilson's disease Autoimmune response causes Primary biliary ...
https://en.wikipedia.org/wiki/Chronic_liver_disease
LeukemiaLeukemia
Table 1.4: Age-Adjusted SEER Incidence and U.S. Death Rates and 5-Year Relative Survival Rates By Primary Cancer Site, Sex and ... myelofibrosis, or the myelodysplastic syndrome. Transient myeloproliferative disease, also termed transient leukemia, involves ... The primary chemotherapeutic plan is combination chemotherapy with chlorambucil or cyclophosphamide, plus a corticosteroid such ...
https://en.wikipedia.org/wiki/Leukemia
International Classification of Diseases for OncologyInternational Classification of Diseases for Oncology
Lymphomatoid papulosis Primary cutaneous anaplastic large cell lymphoma Primary cutaneous CD30+ large T-cell lymphoma M9719/3 ... Acute panmyelosis with myelofibrosis (C42.1) Acute panmyelosis, NOS Acute myelofibrosis Acute myelosclerosis, NOS M9940/3 Hairy ... primary site /6 Malignant, metastatic site Malignant, secondary site /9 Malignant, uncertain whether primary or metastatic site ... uncertain whether primary or metastatic Unclassified tumor, malignant, uncertain whether primary or metastatic M8001/0 Tumor ...
https://en.wikipedia.org/wiki/International_Classification_of_Diseases_for_Oncology
Primary myelofibrosis: MedlinePlus GeneticsPrimary myelofibrosis: MedlinePlus Genetics
Primary myelofibrosis is a condition characterized by the buildup of scar tissue (fibrosis) in the bone marrow, the tissue that ... medlineplus.gov/genetics/condition/primary-myelofibrosis/ Primary myelofibrosis. ... Mutations in the JAK2, MPL, CALR, and TET2 genes are associated with most cases of primary myelofibrosis. The JAK2 and MPL ... Mutations in either the JAK2 gene or the MPL gene that are associated with primary myelofibrosis lead to overactivation of the ...
https://medlineplus.gov/genetics/condition/primary-myelofibrosis
Primary Myelofibrosis Treatment & Management: Approach Considerations, JAK Inhibitors, FGFR InhibitorsPrimary Myelofibrosis Treatment & Management: Approach Considerations, JAK Inhibitors, FGFR Inhibitors
Older terms for this disorder include agnogenic myeloid metaplasia with myelofibrosis and chronic idiopathic myelofibrosis. ... Primary myelofibrosis is a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells. ... encoded search term (Primary Myelofibrosis) and Primary Myelofibrosis What to Read Next on Medscape ... Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis ( ...
https://emedicine.medscape.com/article/197954-treatment
Imetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis - Tabular View - ClinicalTrials.govImetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis - Tabular View - ClinicalTrials.gov
Imetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis. The safety and scientific validity of this ... Imetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis Official Title ICMJE A Pilot Open-Label ... Primary myelofibrosis (PMF) per the revised World Health Organization (WHO) criteria.. *Post-polycythemia vera/essential ... May 24, 2018 (Final data collection date for primary outcome measure) Current Primary Outcome Measures ICMJE (submitted: August ...
https://www.clinicaltrials.gov/ct2/show/record/NCT01731951
Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary MyelofibrosisMayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both ... "New prognostic scoring system for primary myelofibrosis based on a study of the International working group for myelofibrosis ... A. Tefferi, "Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management," American Journal of ... Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both ...
https://www.hindawi.com/journals/crihem/2013/520712/
Fast Five Quiz: Prognosis of Primary MyelofibrosisFast Five Quiz: Prognosis of Primary Myelofibrosis
JAK Inhibitors in Myelofibrosis: Expert Insights on Cases in Personalizing Therapy 1.25 CME / ABIM MOC Credits ...
https://reference.medscape.com/viewarticle/933699_5
Diagnosis and Prognosis of Primary MyelofibrosisDiagnosis and Prognosis of Primary Myelofibrosis
... and symptom burden questionnaires to assess prognosis for patients with myelofibrosis. ... Diagnosis and Prognosis of Primary Myelofibrosis. EP: 2. .Therapeutic Decision-Making for Primary Myelofibrosis. EP: 3. .Recent ... Ruxolitinib for Patients With Primary Myelofibrosis. EP: 6. .Fedratinib for Patients With Primary Myelofibrosis. EP: 7. .Bone ... Diagnosis and Prognosis of Primary Myelofibrosis. Jun 29, 2021. By Ruben A. Mesa, MD, FACP, UT Health San Antonio MD Anderson ...
https://www.onclive.com/view/diagnosis-and-prognosis-of-primary-myelofibrosis
Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia<...
Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia. American journal of ... Dive into the research topics of Blood tests may predict early primary myelofibrosis in patients presenting with essential ... Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia. In: American journal ... Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia. / Carobbio, ...
https://mayoclinic.pure.elsevier.com/en/publications/blood-tests-may-predict-early-primary-myelofibrosis-in-patients-p
FDA Approves Fedratinib for the Treatment of MyelofibrosisFDA Approves Fedratinib for the Treatment of Myelofibrosis
The second drug for myelofibrosis has now been approved, giving patients another option. ... Fedratinib previously received orphan drug designation for the treatment of secondary and primary myelofibrosis and was granted ... of fedratinib as compared to placebo in patients with intermediate-2 or high-risk primary myelofibrosis, post-polycythemia vera ... The primary endpoint of the study was spleen response rate, defined as the proportion of patients who demonstrated a reduction ...
https://www.medscape.com/viewarticle/916928
Bone marrow-specific loss of ABI1 induces myeloproliferative neoplasm with features resembling human myelofibrosisBone marrow-specific loss of ABI1 induces myeloproliferative neoplasm with features resembling human myelofibrosis
Although the pathogenesis of primary myelofibrosis (PMF) and other myeloproliferative neoplasms (MPNs) is linked to ... Although the pathogenesis of primary myelofibrosis (PMF) and other myeloproliferative neoplasms (MPNs) is linked to ... Bone marrow-specific loss of ABI1 induces myeloproliferative neoplasm with features resembling human myelofibrosis Blood. 2018 ...
https://pubmed.ncbi.nlm.nih.gov/30213875/
Primary Myelofibrosis - MD Nexus
Primary Myelofibrosis. Property of Kenneth J. Serio, MD. Author is not responsible for errors in content, site is for ...
https://www.mdnxs.com/topics-2/hematology-oncology/primary-myelofibrosis/
IMSEAR at SEARO: Extramedullary myeloid tumors in primary myelofibrosis.
Extramedullary myeloid tumors in primary myelofibrosis.. Authors: Das, D K. Mohanty, D. Banerjee, A K. Sharma, B K. Jindal, S K ... Extramedullary myeloid tumors in primary myelofibrosis. Indian Journal of Pathology & Microbiology. 1979 Jan; 22(1): 81-4. ...
https://imsear.searo.who.int/handle/123456789/73173?locale=ar
Myelofibrosis-associated complications: pathogenesis, clinical manifes | IJGMMyelofibrosis-associated complications: pathogenesis, clinical manifes | IJGM
Myelofibrosis (MF) is a rare chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)- ... Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes Tariq I Mughal,1 Kris ... New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis ... 8 MF can be primary (termed "primary myelofibrosis" [PMF]; formerly termed "idiopathic MF," "agnogenic myeloid metaplasia," or ...
https://www.dovepress.com/myelofibrosis-associated-complications-pathogenesis-clinical-manifesta-peer-reviewed-fulltext-article-IJGM
Primary Myelofibrosis Pipeline Analysis: 55+ Companies | DelveInsight - Jaipur HeraldPrimary Myelofibrosis Pipeline Analysis: 55+ Companies | DelveInsight - Jaipur Herald
Primary Myelofibrosis Overview. Primary myelofibrosis (also called chronic idiopathic myelofibrosis, agnogenic myeloid ... Primary Myelofibrosis Pipeline Assessment by Stage and Product Type. *Primary Myelofibrosis Pipeline Assessment by Route of ... Primary Myelofibrosis Pipeline Assessment by Stage and Route of Administration. *Primary Myelofibrosis Pipeline Assessment by ... Primary Myelofibrosis Pipeline Assessment by Stage and Molecule Type. *Primary Myelofibrosis Pipeline Companies- Nippon ...
http://www.jaipurherald.in/story/152998/primary-myelofibrosis-pipeline-analysis-55-companies-delveinsight.html
Primary Myelofibrosis Pipeline Analysis: 55+ Companies | DelveInsight - Punjab SamacharPrimary Myelofibrosis Pipeline Analysis: 55+ Companies | DelveInsight - Punjab Samachar
Primary Myelofibrosis Overview. Primary myelofibrosis (also called chronic idiopathic myelofibrosis, agnogenic myeloid ... Primary Myelofibrosis Pipeline Assessment by Stage and Product Type. *Primary Myelofibrosis Pipeline Assessment by Route of ... Primary Myelofibrosis Pipeline Assessment by Stage and Route of Administration. *Primary Myelofibrosis Pipeline Assessment by ... Primary Myelofibrosis Pipeline Assessment by Stage and Molecule Type. *Primary Myelofibrosis Pipeline Companies- Nippon ...
http://www.punjabsamachar.in/story/138304/primary-myelofibrosis-pipeline-analysis-55-companies-delveinsight.html
Secondary Malignancies in Patients with Primary Myelofibrosis - Docwire NewsSecondary Malignancies in Patients with Primary Myelofibrosis - Docwire News
... it is unclear whether patients with primary myelofibrosis (PMF) have the increased risk for second ... it is unclear whether patients with primary myelofibrosis (PMF) have the increased risk for second primary malignancy (SPM) ... Home Conference Coverage ASCO 2022 Secondary Malignancies in Patients with Primary Myelofibrosis ...
https://www.docwirenews.com/conference-coverage/asco2022/secondary-malignancies-in-patients-with-primary-myelofibrosis/
Triple-Negative Primary Myelofibrosis: A Bone Marrow Pathology Group Study. | Mod Pathol;36(3): 100016, 2023 Jan 10. |...Triple-Negative Primary Myelofibrosis: A Bone Marrow Pathology Group Study. | Mod Pathol;36(3): 100016, 2023 Jan 10. |...
Triple-Negative Primary Myelofibrosis: A Bone Marrow Pathology Group Study. Triple-Negative Primary Myelofibrosis: A Bone ... bone marrow pathology; myeloid neoplasms; myeloproliferative neoplasms; primary myelofibrosis; triple-negative primary ... Primary myelofibrosis (PMF) is a clonal myeloproliferative neoplasm driven by canonical gene mutations in JAK2, CALR, or MPL in ...
https://pesquisa.bvsalud.org/controlecancer/resource/pt/mdl-36788093
Appendix H: MDS/MPN Subtypes - Forms Instruction Manual - 1Appendix H: MDS/MPN Subtypes - Forms Instruction Manual - 1
Primary myelofibrosis. *Includes chronic idiopathic myelofibrosis (CIMF), agnogenic2 myeloid metaplasia (AMM), myelofibrosis/ ... Includes primary thrombocytosis, idiopathic thrombocytosis, and hemorrhagic thrombocythemia. *Bone marrow with megakaryocytic ... sclerosis with myeloid metaplasia (MMM), and idiopathic myelofibrosis. *Megakaryocytic hyperplasia with fibrosis (MF 2-3) or ...
https://www.cibmtr.org/manuals/fim/1/en/topic/appendix-h
First Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera - Oncology Nurse AdvisorFirst Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera - Oncology Nurse Advisor
A detailing of the first known case of dermatomyositis associated with secondary myelofibrosis. ... including primary myelofibrosis have been reported. In a case study published in Case Reports in Hematology, investigators ... First Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera Tiffany Garbutt, PhD ... Close more info about First Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera ...
https://www.oncologynurseadvisor.com/home/cancer-types/myeloproliferative-neoplasms/first-case-of-dermatomyositis-associated-with-secondary-myelofibrosis-following-polycythemia-vera/
The role of allogeneic SCT in primary myelofibrosis: a British Society for Blood and Marrow Transplantation study - British...The role of allogeneic SCT in primary myelofibrosis: a British Society for Blood and Marrow Transplantation study - British...
The role of allogeneic SCT in primary myelofibrosis: a British Society for Blood and Marrow Transplantation study. ...
https://bsbmtct.org/publication-archive/the-role-of-allogeneic-sct-in-primary-myelofibrosis-a-british-society-for-blood-and-marrow-transplantation-study/
A population-based study of outcomes in polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the United...A population-based study of outcomes in polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the United...
A population-based study of outcomes in polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the United ...
http://dgivan.dental.uab.edu/display/pub2474521
Media Announcement | News Room | ATSDR
Patients with related conditions, essential thromboycytosis (ET) and primary myelofibrosis, also can carry the mutation. ...
https://wwwn.cdc.gov/TSP/NEWS/NewsDisplay.aspx?newsIndicator=2484
Search Clinical Trials | Vanderbilt-Ingram Cancer CenterSearch Clinical Trials | Vanderbilt-Ingram Cancer Center
Ruxolitinib Phosphate before and after Stem Cell Transplant in Treating Patients with Primary or Secondary Myelofibrosis ... A Study to Evaluate Safety and Efficacy of Selinexor in Combination With Ruxolitinib in Participants With Myelofibrosis ... efficacy of selinexor plus ruxolitinib in treatment nave myelofibrosis (MF) participants.. The study will be conducted in two ... ruxolitinib phosphate before and after stem cell transplant works in treating patients with primary or secondary myelofibrosis ...
https://www.vicc.org/clinical-trials/physicians?keys=&f%5B0%5D=drug_name_physician%3ARuxolitinib
Fundraiser by Loyloy Lagatic : Help Dina fight MyelofibrosisFundraiser by Loyloy Lagatic : Help Dina fight Myelofibrosis
Loyloy Lagatic needs your support for Help Dina fight Myelofibrosis ... she was recently diagnosed with Primary Myelofibrosis (is a disorder of a bone marrow also known as Osteomyelofibrosis ...
https://www.gofundme.com/f/help-dina-fight-myelofibrosis
Vonjo (pacritinib) dosing, indications, interactions, adverse effects, and moreVonjo (pacritinib) dosing, indications, interactions, adverse effects, and more
Myelofibrosis dosing for Vonjo (pacritinib), frequency-based adverse effects, comprehensive interactions, contraindications, ... Myelofibrosis. Indicated for adults with intermediate or high-risk primary or secondary (post-polycythemia vera or post- ... Cytopenic Myelofibrosis: What Is It and How Can We Treat It? 0.25 CME / ABIM MOC Credits ... combined with additional data derived from primary medical literature. ...
https://reference.medscape.com/drug/vonjo-pacritinib-1000150
Cureus | Trametinib: Could It Be a Promising Drug to Treat Atypical Chronic Myeloid Leukemia?Cureus | Trametinib: Could It Be a Promising Drug to Treat Atypical Chronic Myeloid Leukemia?
... primary myelofibrosis; PV: polycythemia vera; ET: essential thrombocythemia. ... primary myelofibrosis; PV: polycythemia vera; ET: essential thrombocythemia. ... Harrison C, Kiladjian JJ, Al-Ali HK, et al.: JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N ... investigated a total of 20 patients (five diagnosed with myelofibrosis, eight diagnosed with CMML, and seven diagnosed with ...
https://www.cureus.com/articles/99290-trametinib-could-it-be-a-promising-drug-to-treat-atypical-chronic-myeloid-leukemia
Claus Werenberg Marcher - Publikationer - Syddansk UniversitetClaus Werenberg Marcher - Publikationer - Syddansk Universitet
Refractory primary immune thrombocytopenia with subsequent del(5q) MDS: Complete remission of both after lenalidomide. Bech ... polycythemia vera and myelofibrosis treated with recombinant interferon alpha. Stauffer Larsen, T., Iversen, K. F., Hansen, E. ...
https://portal.findresearcher.sdu.dk/da/persons/cmarcher/publications/
CN103524509B - The salt of Zhan Nasi kinase inhibitor (R)-3-(4-(7H-pyrrolo-[2,3-d] pyrimidine-4-yl)-1H-pyrazol-1-yl)-3...
208000003476 Primary Myelofibrosis Diseases 0.000 description 1 * 241000288906 Primates Species 0.000 description 1 ...
https://patents.google.com/patent/CN103524509B/en
Acid reflux: treatments, associated drugs and conditions (398,720 reports) - eHealthMe
Primary myelofibrosis: 159 reports. *Primary pulmonary hypertension: 1,625 reports. *Prostate cancer: 315 reports ...
https://www.ehealthme.com/condition/acid-reflux/
PolycythemiaMyeloid metaplasiaRuxolitinibNeoplasmsJAK2ChronicNeoplasmPatient with primary myelofibrosisMutationScar tissue in the bone maSecondaryAcute2022Night sweatsHematologicPrognosisExtramedullaryMutationsSymptomsComplicationsDisorderComplicationAsymptomaticOutcomesSpleenJak1BloodEssentialPatients with primarySumitomo Pharma OncologyAbnormalPathologyTreatmentStagesApproachesRare
Polycythemia20
  • Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT). (medscape.com)
  • We're going to have a discussion, first about myelofibrosis, then about polycythemia vera. (onclive.com)
  • Fedratinib, a highly selective JAK2 inhibitor, is indicated for patients with intermediate-2 or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis. (medscape.com)
  • Today's approval was based on the results of the JAKARTA study, a pivotal phase 3, multicenter, randomized, double-blind, placebo-controlled trial that evaluated the efficacy of daily oral doses (400 mg or 500 mg) of fedratinib as compared to placebo in patients with intermediate-2 or high-risk primary myelofibrosis, post-polycythemia vera myelofibrosis, or post-essential thrombocythemia myelofibrosis with splenomegaly. (medscape.com)
  • In a case study published in Case Reports in Hematology , investigators report on the first known case of DM associated with secondary myelofibrosis (MF) following polycythemia vera (PV) . (oncologynurseadvisor.com)
  • Primary myelofibrosis (also known as chronic idiopathic myelofibrosis) is myelofibrosis that has been diagnosed without any prior MPN's, while secondary myelofibrosis is myelofibrosis that has developed after the patient has first been diagnosed with essential thrombocythemia (ET) or Polycythemia Vera (VR). (massivebio.com)
  • This secondary myelofibrosis can also be known as post-polycythemia vera (PPV) or post-essential thrombocythemia (PET-MF). (massivebio.com)
  • 2) to establish a possible relationship between clinical and laboratory findings in the context of Polycythemia Vera (PV), Essential Thrombocytemia (ET), Primary Myelophibrosis (PMF) and Myeloproliferative Neoplasms unclassifiable (MPN-U). (scirp.org)
  • There are three "classic" types: primary myelofibrosis (MF), essential thrombocythemia (ET) and polycythemia vera (PV). (curetoday.com)
  • 3. With polycythemia vera and essential thrombocytosis, is blood letting the primary treatment? (mayoclinic.org)
  • The four patients who developed lymphomas all had primary (n = 3) or postprimary polycythemia vera myelofibrosis (n = 1) with a JAK2 V617F mutation. (medscape.com)
  • Myeloproliferative neoplasms (MPNs) are chronic hematopoietic stem cell disorders, including polycythemia vera, essential thrombocytosis, and primary myelofibrosis. (elsevier.com)
  • While the exact cause is unknown, scientists believe that mutations, or changes in certain genes, are thought to be a major cause of what are known as Philadelphia chromosome-negative MPNs, or "classical" MPNs, including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). (voicesofmpn.com)
  • Polycythemia vera (PV) is one disease in a group of Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) and is characterized by erythrocytosis, uncontrolled and autonomous hematopoiesis, and evolution to end-stage myelofibrosis or acute nonlymphocytic leukemia. (cdc.gov)
  • The JAK2 V617F is present in 95% to 98% of polycythemia vera (PV), and 50% to 60% of primary myelofibrosis (PMF) and essential thrombocythemia (ET). (marshfieldlabs.org)
  • polycythemia vera, essential thrombocythemia, and primary myelofibrosis, known as classical negative myeloproliferative neoplasms BCR-ABL1 (or Philadelphia chromosome). (bvsalud.org)
  • In particular, overactive JAK signalling is linked to the development of cancer-like conditions called myeloproliferative neoplasms (MPNs) - which include polycythemia vera, essential thrombocythemia and primary myelofibrosis - as well as certain acute childhood leukaemias . (edu.au)
  • Participants with polycythemia vera (PV) or post-polycythemia vera myelofibrosis, ET and MF will receive VAC85135 target dose IM injection with ipilimumab IV infusion at the dose(s) determined by study evaluation team (SET). (who.int)
  • They recently concluded a study which used two major insurance claims databases to identify nearly 300,000 people with essential thrombocythemia, myelofibrosis, and polycythemia vera in the United States. (mpnfoundation.org)
  • The MPN Research Foundation has a single goal: to stimulate original research in pursuit of new treatments - and eventually a cure - for polycythemia vera,essential thrombocythemia and myelofibrosis, known collectively as myeloproliferative neoplasms (MPNs). (mpnfoundation.org)
Myeloid metaplasia8
  • Barosi G. Myelofibrosis with myeloid metaplasia: diagnostic definition and prognostic classification for clinical studies and treatment guidelines. (medscape.com)
  • Agnogenic myeloid metaplasia: a clonal proliferation of hematopoietic stem cells with secondary myelofibrosis. (medscape.com)
  • Primary myelofibrosis (also called chronic idiopathic myelofibrosis, agnogenic myeloid metaplasia) is a disorder in which normal bone marrow tissue is gradually replaced with a fibrous scar-like material. (jaipurherald.in)
  • Purpose: Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and extramedullary hematopoiesis. (theburningofrome.com)
  • Life expectancy in PMF Primary myelofibrosis, also known as idiopathic myelofibrosis or myelofibrosis with myeloid metaplasia, is a rare disease19, 20 usually affecting elderly people. (theburningofrome.com)
  • What are symptoms of myeloid metaplasia with myelofibrosis? (theburningofrome.com)
  • Myeloid metaplasia with myelofibrosis may present in a variety of ways. (theburningofrome.com)
  • A study of 68 patients with symptomatic myelofibrosis and myeloid metaplasia revealed overall response rates of 22% for anemia, 33% for splenomegaly, and 50% for thrombocytopenia. (theburningofrome.com)
Ruxolitinib6
  • Currently, ruxolitinib ( Jakafi, Jakavi, Incyte/Novartis) is the only drug that has been approved for myelofibrosis. (medscape.com)
  • In June 2022, Cellenkos announced that the US Food and Drug Administration (FDA) had cleared its Investigational New Drug (IND) application to initiate a Phase Ib, open-label study of CK0804 as an add-on therapy to ruxolitinib in patients with myelofibrosis who experience a suboptimal response to ruxolitinib. (jaipurherald.in)
  • In June 2022, AbbVie announced new data from Cohort 3 of its Phase II REFINE study of investigational navitoclax in combination with ruxolitinib in JAK inhibitor naïve patients with myelofibrosis (MF), a rare and difficult to treat blood cancer. (jaipurherald.in)
  • This is a global, Phase 1/2, multicenter, open-label study to evaluate the safety and efficacy of selinexor plus ruxolitinib in treatment nave myelofibrosis (MF) participants. (vicc.org)
  • Marked Hyperbilirubinemia Associated with Primary Myelofibrosis Responsive to Ruxolitinib. (ucsf.edu)
  • 4 TP-3654 alone and in combination with ruxolitinib normalized WBC and neutrophil counts, and reduced spleen size and bone marrow fibrosis in JAK2V617F and MPLW515L murine models of myelofibrosis. (massbio.org)
Neoplasms5
  • Although the pathogenesis of primary myelofibrosis (PMF) and other myeloproliferative neoplasms (MPNs) is linked to constitutive activation of the JAK-STAT pathway, JAK inhibitors have neither curative nor MPN-stem cell-eradicating potential, indicating that other targetable mechanisms are contributing to the pathophysiology of MPNs. (nih.gov)
  • Myelofibrosis is part of a group of diseases called myeloproliferative disorders/neoplasms (MPN), and are characterized for abnormal cells which sometimes harbor mutations in the JAK pathway. (massivebio.com)
  • This Philadelphia negative-myeloproliferative neoplasms (MPN) are a spectrum of clonal disorders of the hematopoietic system characterized by overproduction of mature blood elements, a trend to thrombotic and/or hemorrhagic complications with variable rates of transformation to secondary myelofibrosis and acute leukemia [1]. (scirp.org)
  • RUX, a Janus kinase (JAK) 1 and 2 inhibitor that inhibits cytokine signaling and reduces symptoms in myelofibrosis, is increasingly used to treat myeloproliferative neoplasms (MPNs). (ajmc.com)
  • Transcript:Srdan Verstovsek, MD, PhD: Myelofibrosis is one of the myeloproliferative neoplasms, a chronic disease of the bone marrow. (theburningofrome.com)
JAK26
  • Mutations in the JAK2 , MPL , CALR , and TET2 genes are associated with most cases of primary myelofibrosis. (medlineplus.gov)
  • Mutations in either the JAK2 gene or the MPL gene that are associated with primary myelofibrosis lead to overactivation of the JAK/STAT pathway. (medlineplus.gov)
  • Primary myelofibrosis (PMF) is a clonal myeloproliferative neoplasm driven by canonical gene mutations in JAK2, CALR, or MPL in >80% of the cases. (bvsalud.org)
  • The patient was a 56-year-old adult male who was initially diagnosed with V617F JAK2-positive primary myelofibrosis at UZ Leuven, a university hospital in Leuven, Belgium, in 2014. (ajmc.com)
  • My husband was diagnosed at 69, a year ago, with Primary Myelofibrosis including Jak2 mutation. (mayoclinic.org)
  • Las mutaciones somáticas en genes como JAK2, MPL y CARL se comportan como mutaciones drivers iniciadoras, responsables del fenotipo mieloproliferativo. (bvsalud.org)
Chronic2
  • Myelofibrosis (MF) is a rare chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, inefficient hematopoiesis, and shortened survival. (dovepress.com)
  • Myelofibrosis (MF) is a chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative stem cell myeloproliferative neoplasm (MPN) characterized by bone marrow fibrosis, ineffective hematopoiesis, extramedullary hematopoiesis (EMH), splenomegaly, shortened survival and progressive abdominal and constitutional symptoms, as well as other general chronic debilitating complaints. (dovepress.com)
Neoplasm1
  • Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. (hindawi.com)
Patient with primary myelofibrosis1
  • Extramedullary hematopoiesis in the spleen of a patient with primary myelofibrosis. (medscape.com)
Mutation2
  • Some people with primary myelofibrosis do not have a mutation in any of the known genes associated with this condition. (medlineplus.gov)
  • Patients with related conditions, essential thromboycytosis (ET) and primary myelofibrosis, also can carry the mutation. (cdc.gov)
Scar tissue in the bone ma2
  • However, in primary myelofibrosis, the excess collagen forms scar tissue in the bone marrow. (medlineplus.gov)
  • If it occurs as a result of a separate disease, it is known as secondary myelofibrosis (for example, scar tissue in the bone marrow as a complication of an autoimmune disease). (massivebio.com)
Secondary5
  • Secondary endpoints included symptom response rate, defined as the proportion of patients with a ≥50% reduction in total symptom score after six 1-month treatment cycles, as measured by the modified Myelofibrosis Symptom Assessment Form v2.0 diary. (medscape.com)
  • Fedratinib previously received orphan drug designation for the treatment of secondary and primary myelofibrosis and was granted priority review designation. (medscape.com)
  • TP-3654 is currently being evaluated in a Phase 1/2, multicenter, dose-escalation, open-label trial to assess safety, tolerability, pharmacokinetics, and pharmacodynamics in patients with intermediate or high-risk primary or secondary myelofibrosis. (massbio.org)
  • [ 1 ] Bone marrow failure resulting from secondary infiltration is a possible cause of lack of blood cell production (as differentiated from a primary cause of failure). (medscape.com)
  • Secondary myelofibrosis is due to implantation or invasion by malignant cancer cells that have metastasized because of implantation of blood-borne tumor cells from a distant cancer. (medscape.com)
Acute2
  • Common causes of morbidity and mortality include thromboembolic and/or haemorrhagic complications, as well as disease progression to myelofibrosis (MF) and/or transformation to acute myeloid leukaemia (AML), all of which vary in frequencies between MPN subtypes [ 1 ]. (biomedcentral.com)
  • Although portal hypertension has been reportedly associated with myeloproliferative diseases, such as primary myelofibrosis, it has never been reportedly complicated with de novo acute myeloid leukemia. (go.jp)
20225
  • DelveInsight's, " Primary Myelofibrosis Pipeline Insight , 2022" report provides comprehensive insights about 55+ companies and 55+ pipeline drugs in the Primary Myelofibrosis pipeline landscape. (jaipurherald.in)
  • In June 2022, Imago Biosciences presented updated positive data from its ongoing global Phase II clinical study evaluating bomedemstat in patients with advanced myelofibrosis. (jaipurherald.in)
  • In June 2022, announced the US FDA granted Orphan Drug Designation for TP-3654 , Sumitomo Pharma Oncology's proprietary investigational oral inhibitor of PIM kinases, for the treatment of myelofibrosis. (jaipurherald.in)
  • In February 2022, Active Biotech entered into an exclusive license agreement with Oncode Institute in the Netherlands for the global rights to patents relating to the use of tasquinimod and other inhibitors of S100 for use in the treatment of myelofibrosis. (jaipurherald.in)
  • CAMBRIDGE, Mass., June 8, 2022 /PRNewswire/Sumitomo Pharma Oncology, Inc., a clinical-stage company focused on novel cancer therapeutics, today announced the U.S. Food and Drug Administration (FDA) granted Orphan Drug Designation for TP-3654, the company's proprietary investigational oral inhibitor of PIM kinases, for the treatment of myelofibrosis. (massbio.org)
Night sweats2
  • Other common signs and symptoms of primary myelofibrosis include fever, night sweats, and bone pain. (medlineplus.gov)
  • In addition, 36 patients experienced a ≥50% reduction in myelofibrosis-related symptoms, including night sweats, itching, abdominal discomfort, feeling full sooner than normal, pain under the ribs on the left side, and bone or muscle pain. (medscape.com)
Hematologic2
  • Approximately 15% to 30% of DM cases are associated with an underlying malignancy, typically lung and ovarian cancers, but some hematologic malignancies, including primary myelofibrosis have been reported. (oncologynurseadvisor.com)
  • Myelofibrosis is a hematologic condition that predisposes to the formation of large and small portal venous clots. (theburningofrome.com)
Prognosis1
  • A panel of experts in myeloid malignancies begins with a discussion on risk assessment criteria, next-generation sequencing, and symptom burden questionnaires to assess prognosis for patients with myelofibrosis. (onclive.com)
Extramedullary1
  • IMSEAR at SEARO: Extramedullary myeloid tumors in primary myelofibrosis. (who.int)
Mutations3
  • Although mutations in the CALR gene and the TET2 gene are relatively common in primary myelofibrosis, it is unclear how these mutations are involved in the development of the condition. (medlineplus.gov)
  • No primary myelofibrosis patiens (n = 6) harboured TET2 mutations. (scirp.org)
  • Development of Absolute quantification kit for CALR types 1 and 2 mutations for Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) patients, LPI. (kingston.ac.uk)
Symptoms6
  • The shortage of blood cells causes many of the signs and symptoms of primary myelofibrosis. (medlineplus.gov)
  • Initially, most people with primary myelofibrosis have no signs or symptoms. (medlineplus.gov)
  • The symptoms associated with primary myelofibrosis differ and are associated to a build of abnormal blood cells and fibers in the bone marrow. (massivebio.com)
  • Like Myelofibrosis, the symptoms for Myelodysplastic syndrome include fatigue, shortness of breath, skin pallor due to anemia and skin bruising due to low platelets. (massivebio.com)
  • Symptoms of the following disorders can be similar to those of primary myelofibrosis. (theburningofrome.com)
  • Since the cause of primary myelofibrosis is unknown, treatment is directed toward the specific symptoms present in each patient. (theburningofrome.com)
Complications1
  • Over time, myelofibrosis can lead to several complications, including: Increased blood pressure in your liver. (theburningofrome.com)
Disorder3
  • Prior to today, there was one FDA-approved drug to treat patients with myelofibrosis, a rare bone marrow disorder. (medscape.com)
  • My sister Dina, 47 years old, is seeking for your help for her Medical treatment, she was recently diagnosed with Primary Myelofibrosis (is a disorder of a bone marrow also known as Osteomyelofibrosis relatively rare Bone Marrow Cancer)Her Hemoglobin dropped to 4.9 mg/dl few weeks ago presented with Pallor (Pale) and Palpitation with easy fatigability. (gofundme.com)
  • Myelofibrosis (MF) is rare disorder in which abnormal blood cells and fibers build up in the bone marrow. (massivebio.com)
Complication2
  • Anemia is a frequent complication of primary myelofibrosis (PMF), either at presentation or during the course of the disease, with an incidence and diagnosis ranging from 50 to 70% [ 1 ]. (hindawi.com)
  • Mikkael Sekeres, MD, director of the Leukemia Program at the Cleveland Clinic Taussig Cancer Institute, Ohio, commented that this is a "remarkable" study in which the investigators noticed a clinical complication of treatment for myelofibrosis and were able to replicate this complication in mice. (medscape.com)
Asymptomatic2
  • One fourth of patients with primary myelofibrosis are asymptomatic, and the diagnosis is made as a result of detecting splenomegaly or checking blood cell counts for an unrelated cause. (medscape.com)
  • How is primary myelofibrosis treated in asymptomatic patients? (theburningofrome.com)
Outcomes1
  • These data reinforced the importance of early intervention in myelofibrosis and the potential to achieve improved clinical outcomes. (jaipurherald.in)
Spleen3
  • The primary endpoint of the study was spleen response rate, defined as the proportion of patients who demonstrated a reduction in spleen volume of ≥35% after six 1-month treatment cycles. (medscape.com)
  • Most patients with myelofibrosis have an enlarged spleen, and in some cases, an enlarged liver. (massivebio.com)
  • But myelofibrosis might cause them to grow in other parts of your body, like your lungs, liver, spleen, and digestive tract. (theburningofrome.com)
Jak11
  • A subgroup analysis of 216 patients with primary myelofibrosis included 31 treated with JAK1/2 inhibitor therapy. (medscape.com)
Blood9
  • Primary myelofibrosis is a condition characterized by the buildup of scar tissue (fibrosis) in the bone marrow, the tissue that produces blood cells. (medlineplus.gov)
  • Primary myelofibrosis impairs the body's ability to produce normal blood cells. (oncologynurseadvisor.com)
  • Myelofibrosis and Myelodysplastic Syndrome, like low blood counts can be treated with blood transfusions, where red blood cells or platelets are replaced. (massivebio.com)
  • In primary myelofibrosis there are often low ranges of circulating red blood cells, a situation known as anemia. (massivebio.com)
  • In addition, we suggest that deregulation of CDC42 may underlie more common blood disorders, such as primary myelofibrosis. (elsevier.com)
  • Does myelofibrosis cause high blood pressure? (theburningofrome.com)
  • Myelofibrosis is a rare type of bone marrow cancer which disrupts an individual's normal production of blood cells. (massbio.org)
  • Bone marrow is a spongy tissue inside of the bone, and is the primary place where blood cells are made. (voicesofmpn.com)
  • After Margot was diagnosed with myelofibrosis, a blood cancer, she found herself more aware of "all the. (seizethedays.org)
Essential3
  • According to World Health Organization (WHO)-defined criteria, patients presenting clinically as essential thrombocythemia (ET) may show early primary myelofibrosis (PMF) with accompanying thrombocythemia. (elsevier.com)
  • The MPN designation also includes essential thrombocytosis (ET) and primary myelofibrosis (PMF). (cdc.gov)
  • Participants with essential thrombocythemia (ET) and myelofibrosis (MF) will receive VAC85135 target dose intramuscular (IM) injection in the safety lead-in cohort (Cohort 0). (who.int)
Patients with primary6
  • Bleeding is observed in one fourth of patients with primary myelofibrosis and varies in severity from insignificant cutaneous petechiae to severe, life-threatening gastrointestinal (GI) tract bleeding. (medscape.com)
  • Patients with primary myelofibrosis develop osteosclerosis. (medscape.com)
  • One half of patients with primary myelofibrosis have abnormalities of humoral immunity. (medscape.com)
  • Splenomegaly is the most common finding in patients with primary myelofibrosis, and it is present in approximately 90% of patients. (medscape.com)
  • Hepatomegaly is found in 60-70% of patients with primary myelofibrosis, and pallor is observed in 60% of patients. (medscape.com)
  • According to Utsav Joshi and collaborating researchers, it is unclear whether patients with primary myelofibrosis (PMF) have the increased risk for second primary malignancy (SPM) seen in other BCR-ABL-negative myeloproliferative diseases (MPD). (docwirenews.com)
Sumitomo Pharma Oncology1
  • This designation is an important milestone in the development of TP-3654 and highlights the need for potential new treatment options for patients with myelofibrosis," said Patricia S. Andrews, CEO and Global Head of Oncology, Sumitomo Pharma Oncology, Inc. (massbio.org)
Abnormal2
  • In primary myelofibrosis, chemicals released by high numbers of platelets and abnormal megakaryocytes (platelet forming cells) over-stimulate the fibroblasts. (jaipurherald.in)
  • It is related to an abnormal stem cell clone that stimulates increased myelofibrosis and damage. (medscape.com)
Pathology1
  • Triple-Negative Primary Myelofibrosis: A Bone Marrow Pathology Group Study. (bvsalud.org)
Treatment5
  • The US Food and Drug Administration (FDA) has approved fedratinib ( Inrebic , Celgene) capsules for the treatment of adult patients with certain types of myelofibrosis. (medscape.com)
  • We believe fedratinib can play an important role in the treatment of myelofibrosis, and we look forward to working with the FDA as the review process advances. (medscape.com)
  • Cite this: FDA Approves Fedratinib for the Treatment of Myelofibrosis - Medscape - Aug 16, 2019. (medscape.com)
  • DelveInsight's primary myelofibrosis pipeline report depicts a robust space with 50+ active players working to develop 55+ pipeline therapies for primary myelofibrosis treatment. (jaipurherald.in)
  • During a Targeted Oncology case-based roundtable event, Pankit Vachhani, MD, discussed with participants how to assess risk and begin treatment for myelofibrosis. (targetedonc.com)
Stages1
Approaches1
  • The companies and academics are working to assess challenges and seek opportunities that could influence Primary myelofibrosis R&D. The therapies under development are focused on novel approaches to treat/improve Primary myelofibrosis. (jaipurherald.in)
Rare1
  • Primary myelofibrosis is a rare condition that affects approximately 1 in 500,000 people worldwide. (medlineplus.gov)
Imetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis - Tabular View - ClinicalTrials.gov
Imetelstat Sodium in Treating Participants With Primary or Secondary Myelofibrosis - Tabular View - ClinicalTrials.gov (clinicaltrials.gov)
Appendix H: MDS/MPN Subtypes - Forms Instruction Manual - 1
Appendix H: MDS/MPN Subtypes - Forms Instruction Manual - 1 (cibmtr.org)
Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis
Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis (hindawi.com)
Search Clinical Trials | Vanderbilt-Ingram Cancer Center
Search Clinical Trials | Vanderbilt-Ingram Cancer Center (vicc.org)
Myelofibrosis-associated complications: pathogenesis, clinical manifes | IJGM
Myelofibrosis-associated complications: pathogenesis, clinical manifes | IJGM (dovepress.com)
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Fundraiser by Loyloy Lagatic : Help Dina fight Myelofibrosis (gofundme.com)
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Vonjo (pacritinib) dosing, indications, interactions, adverse effects, and more (reference.medscape.com)
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Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia<... (mayoclinic.pure.elsevier.com)
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Vol. 98 No. 11 (2013): November, 2013 | Haematologica (haematologica.org)
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Hunza Chaudhry | UCSF Profiles (profiles.ucsf.edu)
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TET2 Mutations in Ph-Negative-Myeloproliferative Neoplasms: Identification of Three Novel Mutations and Relationship with... (file.scirp.org)
JAK Inhibitors: Uses, Drug Options, and Side Effects
JAK Inhibitors: Uses, Drug Options, and Side Effects (verywellhealth.com)
Acquired platelet function defect: MedlinePlus Medical Encyclopedia
Acquired platelet function defect: MedlinePlus Medical Encyclopedia (medlineplus.gov)
December 2017
December 2017 (newsnetwork.mayoclinic.org)
First Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera - Oncology Nurse Advisor
First Case of Dermatomyositis Associated with Secondary Myelofibrosis Following Polycythemia Vera - Oncology Nurse Advisor (oncologynurseadvisor.com)
Clonal hematopoietic mutations linked to platelet traits and the risk of thrombosis or bleeding | Haematologica
Clonal hematopoietic mutations linked to platelet traits and the risk of thrombosis or bleeding | Haematologica (haematologica.org)
JAK2 Mutation - Effects and Questions | Page 15 | Mayo Clinic Connect
JAK2 Mutation - Effects and Questions | Page 15 | Mayo Clinic Connect (connect.mayoclinic.org)
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ZTE Spro 2 Review - Photography Life (photographylife.com)
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Sphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia |... (haematologica.org)
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Search Clinical Trials | Froedtert & MCW (froedtert.com)