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.
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.
A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
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.
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.
Cell surface receptors that are specific for THROMBOPOIETIN. They signal through interaction with JANUS KINASES such as JANUS KINASE 2.
Enlargement of the spleen.
Clonal myeloid disorders that possess both dysplastic and proliferative features but are not properly classified as either MYELODYSPLASTIC SYNDROMES or MYELOPROLIFERATIVE DISORDERS.
Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)
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.
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.
Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.
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.
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.
Mapping of the KARYOTYPE of a cell.
The formation and development of blood cells outside the BONE MARROW, as in the SPLEEN; LIVER; or LYMPH NODES.
The number of PLATELETS per unit volume in a sample of venous BLOOD.
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.
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)
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.
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.
An abnormal hardening or increased density of bone tissue.
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.
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.

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)

Primary myelofibrosis (PMF) is a rare, chronic bone marrow disorder characterized by the replacement of normal bone marrow tissue with fibrous scar tissue, leading to impaired production of blood cells. This results in cytopenias (anemia, leukopenia, thrombocytopenia), which can cause fatigue, infection susceptibility, and bleeding tendencies. Additionally, PMF is often accompanied by the proliferation of abnormal megakaryocytes (large, atypical bone marrow cells that produce platelets) and extramedullary hematopoiesis (blood cell formation outside the bone marrow, typically in the spleen and liver).

PMF is a type of myeloproliferative neoplasm (MPN), which is a group of clonal stem cell disorders characterized by excessive proliferation of one or more types of blood cells. PMF can present with various symptoms such as fatigue, weight loss, night sweats, abdominal discomfort due to splenomegaly (enlarged spleen), and bone pain. In some cases, PMF may progress to acute myeloid leukemia (AML).

The exact cause of PMF remains unclear; however, genetic mutations are known to play a significant role in its development. The Janus kinase 2 (JAK2), calreticulin (CALR), and MPL genes have been identified as commonly mutated in PMF patients. These genetic alterations contribute to the dysregulated production of blood cells and the activation of signaling pathways that promote fibrosis.

Diagnosis of PMF typically involves a combination of clinical evaluation, complete blood count (CBC), bone marrow aspiration and biopsy, cytogenetic analysis, and molecular testing to identify genetic mutations. Treatment options depend on the individual patient's symptoms, risk stratification, and disease progression. They may include observation, supportive care, medications to manage symptoms and control the disease (such as JAK inhibitors), and stem cell transplantation for eligible patients.

Janus Kinase 2 (JAK2) is a tyrosine kinase enzyme that plays a crucial role in intracellular signal transduction. It is named after the Roman god Janus, who is depicted with two faces, as JAK2 has two similar phosphate-transferring domains. JAK2 is involved in various cytokine receptor-mediated signaling pathways and contributes to hematopoiesis, immune function, and cell growth.

Mutations in the JAK2 gene have been associated with several myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The most common mutation is JAK2 V617F, which results in a constitutively active enzyme that promotes uncontrolled cell proliferation and survival, contributing to the development of these MPNs.

Essential thrombocythemia (ET) is a myeloproliferative neoplasm (MPN), a type of blood cancer characterized by the overproduction of platelets (thrombocytosis) in the bone marrow. In ET, there is an excessive proliferation of megakaryocytes, the precursor cells that produce platelets. This leads to increased platelet counts in the peripheral blood, which can increase the risk of blood clots (thrombosis) and bleeding episodes (hemorrhage).

The term "essential" is used to indicate that the cause of this condition is not known or idiopathic. ET is primarily a disease of older adults, but it can also occur in younger individuals. The diagnosis of essential thrombocythemia requires careful evaluation and exclusion of secondary causes of thrombocytosis, such as reactive conditions, inflammation, or other myeloproliferative neoplasms.

The clinical presentation of ET can vary widely among patients. Some individuals may be asymptomatic and discovered only during routine blood tests, while others may experience symptoms related to thrombosis or bleeding. Common symptoms include headaches, visual disturbances, dizziness, weakness, numbness, or tingling in the extremities, if there are complications due to blood clots in the brain or other parts of the body. Excessive bruising, nosebleeds, or blood in the stool can indicate bleeding complications.

Treatment for essential thrombocythemia is aimed at reducing the risk of thrombosis and managing symptoms. Hydroxyurea is a commonly used medication to lower platelet counts, while aspirin may be prescribed to decrease the risk of blood clots. In some cases, interferon-alpha or ruxolitinib might be considered as treatment options. Regular follow-up with a hematologist and monitoring of blood counts are essential for managing this condition and detecting potential complications early.

Polycythemia Vera is a type of myeloproliferative neoplasm, a group of rare blood cancers. In Polycythemia Vera, the body produces too many red blood cells, leading to an increased risk of blood clots and thickening of the blood, which can cause various symptoms such as fatigue, headache, dizziness, and itching. It can also lead to enlargement of the spleen. The exact cause of Polycythemia Vera is not known, but it is associated with genetic mutations in the JAK2 gene in most cases. It is a progressive disease that can lead to complications such as bleeding, thrombosis, and transformation into acute leukemia if left untreated.

Myeloproliferative disorders (MPDs) are a group of rare, chronic blood cancers that originate from the abnormal proliferation or growth of one or more types of blood-forming cells in the bone marrow. These disorders result in an overproduction of mature but dysfunctional blood cells, which can lead to serious complications such as blood clots, bleeding, and organ damage.

There are several subtypes of MPDs, including:

1. Chronic Myeloid Leukemia (CML): A disorder characterized by the overproduction of mature granulocytes (a type of white blood cell) in the bone marrow, leading to an increased number of these cells in the blood. CML is caused by a genetic mutation that results in the formation of the BCR-ABL fusion protein, which drives uncontrolled cell growth and division.
2. Polycythemia Vera (PV): A disorder characterized by the overproduction of all three types of blood cells - red blood cells, white blood cells, and platelets - in the bone marrow. This can lead to an increased risk of blood clots, bleeding, and enlargement of the spleen.
3. Essential Thrombocythemia (ET): A disorder characterized by the overproduction of platelets in the bone marrow, leading to an increased risk of blood clots and bleeding.
4. Primary Myelofibrosis (PMF): A disorder characterized by the replacement of normal bone marrow tissue with scar tissue, leading to impaired blood cell production and anemia, enlargement of the spleen, and increased risk of infections and bleeding.
5. Chronic Neutrophilic Leukemia (CNL): A rare disorder characterized by the overproduction of neutrophils (a type of white blood cell) in the bone marrow, leading to an increased number of these cells in the blood. CNL can lead to an increased risk of infections and organ damage.

MPDs are typically treated with a combination of therapies, including chemotherapy, targeted therapy, immunotherapy, and stem cell transplantation. The choice of treatment depends on several factors, including the subtype of MPD, the patient's age and overall health, and the presence of any comorbidities.

Thrombopoietin receptors are a type of cell surface receptor found on megakaryocytes and platelets. They are also known as MPL (myeloproliferative leukemia virus) receptors. Thrombopoietin is a hormone that regulates the production of platelets in the body, and it binds to these receptors to stimulate the proliferation and differentiation of megakaryocytes, which are large bone marrow cells that produce platelets.

The thrombopoietin receptor is a type I transmembrane protein with an extracellular domain that contains the thrombopoietin-binding site, a single transmembrane domain, and an intracellular domain that contains several tyrosine residues that become phosphorylated upon thrombopoietin binding. This triggers a signaling cascade that leads to the activation of various downstream pathways involved in cell proliferation, differentiation, and survival.

Mutations in the thrombopoietin receptor gene have been associated with certain myeloproliferative neoplasms, such as essential thrombocythemia and primary myelofibrosis, which are characterized by excessive platelet production and bone marrow fibrosis.

Splenomegaly is a medical term that refers to an enlargement or expansion of the spleen beyond its normal size. The spleen is a vital organ located in the upper left quadrant of the abdomen, behind the stomach and below the diaphragm. It plays a crucial role in filtering the blood, fighting infections, and storing red and white blood cells and platelets.

Splenomegaly can occur due to various underlying medical conditions, including infections, liver diseases, blood disorders, cancer, and inflammatory diseases. The enlarged spleen may put pressure on surrounding organs, causing discomfort or pain in the abdomen, and it may also lead to a decrease in red and white blood cells and platelets, increasing the risk of anemia, infections, and bleeding.

The diagnosis of splenomegaly typically involves a physical examination, medical history, and imaging tests such as ultrasound, CT scan, or MRI. Treatment depends on the underlying cause and may include medications, surgery, or other interventions to manage the underlying condition.

Myelodysplastic-myeloproliferative diseases (MDS/MPD) are a group of rare and complex bone marrow disorders that exhibit features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN). MDS is characterized by ineffective hematopoiesis, leading to cytopenias, and dysplastic changes in the bone marrow. MPNs are clonal disorders of the hematopoietic stem cells resulting in increased proliferation of one or more cell lines, often leading to elevated blood counts.

MDS/MPD share features of both these entities, with patients showing signs of both ineffective hematopoiesis and increased cell production. These disorders have overlapping clinical, laboratory, and morphological characteristics, making their classification challenging. The World Health Organization (WHO) has recognized several MDS/MPD subtypes, including chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), atypical chronic myeloid leukemia (aCML), and myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS/MPN, U).

The pathogenesis of MDS/MPD involves genetic mutations that affect various cellular processes, such as signal transduction, epigenetic regulation, and splicing machinery. The prognosis for patients with MDS/MPD varies depending on the specific subtype, age, performance status, and the presence of certain genetic abnormalities. Treatment options may include supportive care, chemotherapy, targeted therapy, or stem cell transplantation.

Thrombocytosis is a medical condition characterized by an abnormally high platelet count (also known as thrombocytes) in the blood. Platelets are small cell fragments that play a crucial role in blood clotting. A normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. Thrombocytosis is typically defined as a platelet count exceeding 450,000-500,000 platelets/µL.

Thrombocytosis can be classified into two types: reactive (or secondary) thrombocytosis and primary (or essential) thrombocytosis. Reactive thrombocytosis is more common and occurs as a response to an underlying condition, such as infection, inflammation, surgery, or certain types of cancer. Primary thrombocytosis, on the other hand, is caused by intrinsic abnormalities in the bone marrow cells responsible for platelet production (megakaryocytes), and it is often associated with myeloproliferative neoplasms like essential thrombocythemia.

While mild thrombocytosis may not cause any symptoms, higher platelet counts can increase the risk of blood clots (thrombosis) and bleeding disorders due to excessive platelet aggregation. Symptoms of thrombocytosis may include headaches, dizziness, visual disturbances, or chest pain if a blood clot forms in the brain or heart. Bleeding symptoms can manifest as easy bruising, nosebleeds, or gastrointestinal bleeding.

Treatment for thrombocytosis depends on the underlying cause and the severity of the condition. In cases of reactive thrombocytosis, treating the underlying disorder often resolves the high platelet count. For primary thrombocytosis, medications like aspirin or cytoreductive therapy (such as hydroxyurea) may be used to reduce the risk of blood clots and control platelet production. Regular monitoring of platelet counts is essential for managing this condition and preventing potential complications.

Sea-Blue Histiocyte Syndrome is a rare, inherited disorder characterized by the accumulation of abnormal histiocytes (a type of white blood cell) in various organs and tissues of the body. The histiocytes have a distinctive appearance, with small vacuoles or "blebs" that give them a foamy or bubbly appearance under the microscope, leading to the name "Sea-Blue."

The syndrome is typically diagnosed in childhood or adolescence and is often associated with neurological symptoms such as ataxia (loss of coordination), seizures, and developmental delay. Other features may include anemia, splenomegaly (enlarged spleen), and bone changes leading to fractures.

Sea-Blue Histiocyte Syndrome is caused by mutations in the SPTPS gene, which provides instructions for making a protein involved in the production of lysosomes, structures inside cells that help break down waste products. The genetic defect leads to an accumulation of lipids and other substances within the histiocytes, causing their characteristic appearance.

Treatment for Sea-Blue Histiocyte Syndrome is generally supportive and aimed at managing symptoms as they arise. This may include physical therapy, medications to control seizures or neurological symptoms, and orthopedic interventions for bone fractures. In some cases, stem cell transplantation may be considered as a treatment option.

Bone marrow is the spongy tissue found inside certain bones in the body, such as the hips, thighs, and vertebrae. It is responsible for producing blood-forming cells, including red blood cells, white blood cells, and platelets. There are two types of bone marrow: red marrow, which is involved in blood cell production, and yellow marrow, which contains fatty tissue.

Red bone marrow contains hematopoietic stem cells, which can differentiate into various types of blood cells. These stem cells continuously divide and mature to produce new blood cells that are released into the circulation. Red blood cells carry oxygen throughout the body, white blood cells help fight infections, and platelets play a crucial role in blood clotting.

Bone marrow also serves as a site for immune cell development and maturation. It contains various types of immune cells, such as lymphocytes, macrophages, and dendritic cells, which help protect the body against infections and diseases.

Abnormalities in bone marrow function can lead to several medical conditions, including anemia, leukopenia, thrombocytopenia, and various types of cancer, such as leukemia and multiple myeloma. Bone marrow aspiration and biopsy are common diagnostic procedures used to evaluate bone marrow health and function.

Megakaryocytes are large, specialized bone marrow cells that are responsible for the production and release of platelets (also known as thrombocytes) into the bloodstream. Platelets play an essential role in blood clotting and hemostasis, helping to prevent excessive bleeding during injuries or trauma.

Megakaryocytes have a unique structure with multilobed nuclei and abundant cytoplasm rich in organelles called alpha-granules and dense granules, which store various proteins, growth factors, and enzymes necessary for platelet function. As megakaryocytes mature, they extend long cytoplasmic processes called proplatelets into the bone marrow sinuses, where these extensions fragment into individual platelets that are released into circulation.

Abnormalities in megakaryocyte number, size, or function can lead to various hematological disorders, such as thrombocytopenia (low platelet count), thrombocytosis (high platelet count), and certain types of leukemia.

A mutation is a permanent change in the DNA sequence of an organism's genome. Mutations can occur spontaneously or be caused by environmental factors such as exposure to radiation, chemicals, or viruses. They may have various effects on the organism, ranging from benign to harmful, depending on where they occur and whether they alter the function of essential proteins. In some cases, mutations can increase an individual's susceptibility to certain diseases or disorders, while in others, they may confer a survival advantage. Mutations are the driving force behind evolution, as they introduce new genetic variability into populations, which can then be acted upon by natural selection.

Prognosis is a medical term that refers to the prediction of the likely outcome or course of a disease, including the chances of recovery or recurrence, based on the patient's symptoms, medical history, physical examination, and diagnostic tests. It is an important aspect of clinical decision-making and patient communication, as it helps doctors and patients make informed decisions about treatment options, set realistic expectations, and plan for future care.

Prognosis can be expressed in various ways, such as percentages, categories (e.g., good, fair, poor), or survival rates, depending on the nature of the disease and the available evidence. However, it is important to note that prognosis is not an exact science and may vary depending on individual factors, such as age, overall health status, and response to treatment. Therefore, it should be used as a guide rather than a definitive forecast.

Karyotyping is a medical laboratory test used to study the chromosomes in a cell. It involves obtaining a sample of cells from a patient, usually from blood or bone marrow, and then staining the chromosomes so they can be easily seen under a microscope. The chromosomes are then arranged in pairs based on their size, shape, and other features to create a karyotype. This visual representation allows for the identification and analysis of any chromosomal abnormalities, such as extra or missing chromosomes, or structural changes like translocations or inversions. These abnormalities can provide important information about genetic disorders, diseases, and developmental problems.

Extramedullary hematopoiesis (EMH) is defined as the production of blood cells outside of the bone marrow in adults. In normal physiological conditions, hematopoiesis occurs within the bone marrow cavities of flat bones such as the pelvis, ribs, skull, and vertebrae. However, certain disease states or conditions can cause EMH to occur in various organs such as the liver, spleen, lymph nodes, and peripheral blood.

EMH can be seen in several pathological conditions, including hematologic disorders such as myeloproliferative neoplasms (e.g., polycythemia vera, essential thrombocytopenia), myelodysplastic syndromes, and leukemias. It can also occur in response to bone marrow failure or infiltration by malignant cells, as well as in some non-hematologic disorders such as fibrocystic disease of the breast and congenital hemolytic anemias.

EMH may lead to organ enlargement, dysfunction, and clinical symptoms depending on the site and extent of involvement. Treatment of EMH is generally directed at managing the underlying condition causing it.

A platelet count is a laboratory test that measures the number of platelets, also known as thrombocytes, in a sample of blood. Platelets are small, colorless cell fragments that circulate in the blood and play a crucial role in blood clotting. They help to stop bleeding by sticking together to form a plug at the site of an injured blood vessel.

A normal platelet count ranges from 150,000 to 450,000 platelets per microliter (µL) of blood. A lower than normal platelet count is called thrombocytopenia, while a higher than normal platelet count is known as thrombocytosis.

Abnormal platelet counts can be a sign of various medical conditions, including bleeding disorders, infections, certain medications, and some types of cancer. It is important to consult with a healthcare provider if you have any concerns about your platelet count or if you experience symptoms such as easy bruising, prolonged bleeding, or excessive menstrual flow.

The World Health Organization (WHO) is not a medical condition or term, but rather a specialized agency of the United Nations responsible for international public health. Here's a brief description:

The World Health Organization (WHO) is a specialized agency of the United Nations that acts as the global authority on public health issues. Established in 1948, WHO's primary role is to coordinate and collaborate with its member states to promote health, prevent diseases, and ensure universal access to healthcare services. WHO is headquartered in Geneva, Switzerland, and has regional offices around the world. It plays a crucial role in setting global health standards, monitoring disease outbreaks, and providing guidance on various public health concerns, including infectious diseases, non-communicable diseases, mental health, environmental health, and maternal, newborn, child, and adolescent health.

A missense mutation is a type of point mutation in which a single nucleotide change results in the substitution of a different amino acid in the protein that is encoded by the affected gene. This occurs when the altered codon (a sequence of three nucleotides that corresponds to a specific amino acid) specifies a different amino acid than the original one. The function and/or stability of the resulting protein may be affected, depending on the type and location of the missense mutation. Missense mutations can have various effects, ranging from benign to severe, depending on the importance of the changed amino acid for the protein's structure or function.

Reticulin is a type of protein fiber that forms part of the extracellular matrix in various connective tissues in the body. It is composed of collagenous and non-collagenous proteins, and it has a reticular or network-like structure when viewed under a microscope. In histology (the study of the microscopic structure of tissues), reticulin fibers are often stained to help identify certain types of cells or structures.

In particular, reticulin fibers are often found in close association with certain types of cells, such as hematopoietic stem cells and neurons. They provide structural support and help regulate the function of these cells. In addition, reticulin fibers play a role in the immune response, wound healing, and tissue repair.

Abnormal accumulations of reticulin fibers can be seen in various disease states, such as fibrosis (excessive scarring) and certain types of cancer. For example, increased reticulin fibers are often found in the liver in patients with cirrhosis, a condition characterized by extensive scarring and damage to the liver. Similarly, abnormal reticulin fiber deposition is seen in some forms of lymphoma, a type of cancer that affects the lymphatic system.

An amino acid substitution is a type of mutation in which one amino acid in a protein is replaced by another. This occurs when there is a change in the DNA sequence that codes for a particular amino acid in a protein. The genetic code is redundant, meaning that most amino acids are encoded by more than one codon (a sequence of three nucleotides). As a result, a single base pair change in the DNA sequence may not necessarily lead to an amino acid substitution. However, if a change does occur, it can have a variety of effects on the protein's structure and function, depending on the nature of the substituted amino acids. Some substitutions may be harmless, while others may alter the protein's activity or stability, leading to disease.

Osteosclerosis is a medical term that refers to an abnormal thickening and increased density of bone tissue. This condition can occur as a result of various diseases or conditions, such as certain types of bone cancer, Paget's disease of bone, fluoride poisoning, or chronic infection of the bone. Osteosclerosis can also be seen in some benign conditions, such as osteopetrosis, which is a rare genetic disorder characterized by an excessively hard and dense skeleton.

In some cases, osteosclerosis may not cause any symptoms and may only be discovered on X-rays or other imaging studies. However, in other cases, it can lead to complications such as bone pain, fractures, or deformities. Treatment for osteosclerosis depends on the underlying cause of the condition and may include medications, surgery, or other therapies.

Disease progression is the worsening or advancement of a medical condition over time. It refers to the natural course of a disease, including its development, the severity of symptoms and complications, and the impact on the patient's overall health and quality of life. Understanding disease progression is important for developing appropriate treatment plans, monitoring response to therapy, and predicting outcomes.

The rate of disease progression can vary widely depending on the type of medical condition, individual patient factors, and the effectiveness of treatment. Some diseases may progress rapidly over a short period of time, while others may progress more slowly over many years. In some cases, disease progression may be slowed or even halted with appropriate medical interventions, while in other cases, the progression may be inevitable and irreversible.

In clinical practice, healthcare providers closely monitor disease progression through regular assessments, imaging studies, and laboratory tests. This information is used to guide treatment decisions and adjust care plans as needed to optimize patient outcomes and improve quality of life.

Medical survival rate is a statistical measure used to determine the percentage of patients who are still alive for a specific period of time after their diagnosis or treatment for a certain condition or disease. It is often expressed as a five-year survival rate, which refers to the proportion of people who are alive five years after their diagnosis. Survival rates can be affected by many factors, including the stage of the disease at diagnosis, the patient's age and overall health, the effectiveness of treatment, and other health conditions that the patient may have. It is important to note that survival rates are statistical estimates and do not necessarily predict an individual patient's prognosis.

... 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. ... It is indicated for the treatment of intermediate or high-risk myelofibrosis, including primary myelofibrosis or secondary ... Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a ...
"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 ...
Like pre-PMF, overt primary myelofibrosis is associated with JAK2, CALR, or MPL mutations. However, a bone marrow biopsy will ... In rare cases, some MPNs such as primary myelofibrosis may accelerate and turn into acute myeloid leukemia. MPNs are classified ... "What is Prefibrotic Primary Myelofibrosis?". Patient Power. HealthCentral LLC. Retrieved 23 June 2023. "Summary". www. ... Recently, a JAK2 inhibitor, namely ruxolitinib, has been approved for use in primary myelofibrosis. Trials of these inhibitors ...
"MicroRNA expression profile in granulocytes from primary myelofibrosis patients". Experimental Hematology. 35 (11): 1708-18. ...
"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 ...
"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 ...
... 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 ...
... primary myelofibrosis. Albert Ndele, 92, Congolese economist and politician, chairman of the board of commissioners-general ( ...
January 2009). "CD146(+) bone marrow osteoprogenitors increase in the advanced stages of primary myelofibrosis". Haematologica ...
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 ...
Miller died from primary myelofibrosis on 1 April 2023, at the age of 75. Green, Antony (2008). "Retiring MPs". 2008 Northern ...
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- ...
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 ...
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 ...
"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" (Press ... 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 ...
... and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092-7. doi:10.1182/ ...
... also leads to hematological disruption in mice resulting in a myeloproliferative neoplasm resembling primary myelofibrosis ...
... 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 ...
... primary myelofibrosis, chronic neutrophilic leukemia , and chronic eosinophilic leukemia. This article includes a list of ...
... and primary myelofibrosis show an inherent tendency for transformation into leukemia (MPN-blast phase), which is accompanied by ... primary tumor specimens by use of affymetrix single-nucleotide-polymorphism genotyping microarrays". Am J Hum Genet. 81 (1): ... a medulloblastoma with an isochromosome 17q from a primary rhabdoid tumor with loss of 22q11.2. When indicated, molecular ...
... a US government fellowship Primary myelofibrosis, a disease affecting the bone marrow. Probability mass function, in statistics ...
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 ...
... 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 ...
... 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 ...
... 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 one of the blood cancers wherein the bone ... Branehog I, Ridell B, Swolin B, Weinfeld A (1975). "Megakaryocyte quantifications in relation to thrombokinetics in primary ...
... 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 ...
... 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 - ...
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 ...
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 ...
... deficiency Alcoholism Myelodysplastic syndromes Sideroblastic anemia Congenital dyserythropoietic anemia Primary 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. ... It is indicated for the treatment of intermediate or high-risk myelofibrosis, including primary myelofibrosis or secondary ... Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a ...
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 ...
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 ( ...
Proudly Served by LiteSpeed Web Server at www.scivee.tv Port ...
... is an alternative cure for myelofibrosis (MF), we evaluated 35 UCBTs reported to Eurocord. Seven patients had secondary acute ... Unrelated cord blood transplantation for patients with primary or secondary myelofibrosis Biol Blood Marrow Transplant. 2014 ... To determine whether umbilical cord blood transplantation (UCBT) is an alternative cure for myelofibrosis (MF), we evaluated 35 ...
... , also known as idiopathic myelofibrosis and agnogenic myeloid metaplasia, is a malignant disease, one of ... Primary myelofibrosis is characterised by the presence of a fibrous tissue in the bone marrow, and by the migration of bone ... Links of interest about other general issues that may be of interest to patients with primary myelofibrosis. *. ... develop symptoms of bone marrow fibrosis indistinguishable from that in primary myelofibrosis. ...
Primary Myelofibrosis - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical ... Prognosis for Primary Myelofibrosis The median survival in primary myelofibrosis is 5 years from onset, but variation is wide; ... gene also may be the cause of primary myelofibrosis. However, there are rare cases of primary myelofibrosis in which none of ... Pathophysiology of Primary Myelofibrosis Myelofibrosis is a reactive, reversible increase in bone marrow collagen often with ...
Constellation of images showing various findings of Primary Myelofibrosis. ... Primary Myelofibrosis. Author: Deepika V, MD, 09/09/2022 Category: Myeloid Neoplasms and acute leukemia (WHO 2016) > ...
A case study involving a 72-year-old elementary school teacher been presented with the symptoms of Primary Myelofibrosis. Learn ...
Online resource for healthcare professionals, providing information, news & support relating to MPN
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 ( ...
... is a type of Primary Myelofibrosis. title Primary Myelofibrosis,Overt Fibrotic Stage ... primary-myelofibrosis.html · primary-myelofibrosisovert-fibrotic-stage.html · primary-neuroepithelial-tumor.html. ...
Article by David Wallace / Blog, Essential Thrombocythemia / essential thrombocythemia, ET, pre-fibrotic, primary myelofibrosis ... Abstract In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was ... Jean Jacques Kiladjian ASH 2018 RuxoPeg Phase 1/2 Trial of the Combo of Ruxolitinib and Pegasys in Myelofibrosis Patients ... Michael Grunwald ASH 2018 Risk Assessment and Treatment Myelofibrosis Patients at Community Oncology Practices in the U.S. ...
... ... Purpose To develop a prognostic system for transplantation-age patients with primary myelofibrosis (PMF) that integrates ... Purpose To develop a prognostic system for transplantation-age patients with primary myelofibrosis (PMF) that integrates ...
The Myelofibrosis Appointment Guide helps you ask the right questions at your next doctors appointment. ... Chronic Myeloproliferative Neoplasms Treatment (PDQ®)-Health Professional Version: Primary Myelofibrosis. National Cancer ... What is your main concern about a myelofibrosis diagnosis? * Are you concerned how myelofibrosis may affect other health ... Myelofibrosis Appointment Guide Ask the right questions at your next doctors appointment. Answer two questions below to ...
Treatment of primary myelofibrosis in patients without symptoms is usually watchful waiting. Learn more about treatments for ... Treatment of primary myelofibrosis in patients with symptoms may include the following:. *Transfusion of red blood cells to ... Treatment of primary myelofibrosis in patients without symptoms is usually watchful waiting. ...
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Chronic myeloid leukemia mimicking primary myelofibrosis: A case report Anju S*, Jayalakshmy PL and Sankar Sundaram. Bone ... of Chronic myeloid leukemia in fibrosis and blast crisis in a 32 year old lady which closely resembled Primary myelofibrosis in ... and often associated with blast crisis has previously been reported in both Chronic myeloid leukemia and Primary myelofibrosis ...
Learn Primary Myelofibrosis - Other Hematologic Pathology - Pathology - Picmonic for Medicine faster and easier with Picmonics ... Primary myelofibrosis (PMF) is a type of cancer of bone marrow and is characterized by bone marrow fibrosis, extramedullary ... Primary Myelofibrosis. Quiz. Picmonics rapid review multiple-choice quiz allows you to assess your knowledge. ... Peripheral blood smear of patients with primary myelofibrosis can show dacrocytes, which results from red blood cells squeezing ...
... we provide updated data on ruxolitinib therapy for patients with myelofibrosis and offer expert opinion on the appropriate use ... New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis ... 8. Mascarenhas J, Hoffman R. Risk adapted approach to the treatment of primary myelofibrosis. In : the education program for ... 32. Santos FP, Verstovsek S. What is next beyond Janus kinase 2 inhibitors for primary myelofibrosis? Curr Opin Hematol. 2013; ...
GATA1 downregulation in prefibrotic and fibrotic stages of primary myelofibrosis and in the myelofibrotic progression of other ... GATA1 downregulation in prefibrotic and fibrotic stages of primary myelofibrosis and in the myelofibrotic progression of other ... to impaired hematopoiesis and bone marrow fibrosis in murine models and in vivo in patients affected by primary myelofibrosis ( ...
prefibrotic primary myelofibrosis (pre-PMF) / essential thrombocythemia (ET) / differentialdiagnosis / predictive model. Source ... Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation ... Easily Applicable Predictive Score for Differential Diagnosis of Prefibrotic Primary Myelofibrosis from Essential ... and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. The aim of ...
New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis ... Barosi G, Poletto V, Massa M. JAK2 V617F genotype is a strong determinant of blast transformation in primary myelofibrosis. ... Vannucchi AM, Lasho TL, Guglielmelli P. Mutations and prognosis in primary myelofibrosis. Leukemia. 2013; 27(9):1861-1869. ... Recombinant interferon-α may retard progression of early primary myelofibrosis: a preliminary report. Blood. 2011; 117(24):6669 ...
... Guglielmelli, P; ... classification of myeloproliferative neoplasms defines 2 stages of primary myelofibrosis (PMF) that is, prefibrotic/early (pre- ... classification of myeloproliferative neoplasms defines 2 stages of primary myelofibrosis (PMF) that is, prefibrotic/early (pre- ...
Essential thrombocythemia and primary myelofibrosis. 7718 Immune thrombocytopenia. 7705 Multiple myeloma. 7712 ...
Primary myelofibrosis *Myelodysplastic syndromes *Other myeloid neoplasms. *Presence of JAK2, CALR, or MPL mutation ...
Primary myelofibrosis.. *Post-polycythemia vera myelofibrosis.. *Post-essential thrombocythemia myelofibrosis.. Pacritinib ... Myelofibrosis (a bone marrow disease) that is intermediate or high risk with a very low platelet count, including the following ...
PERSIST-1: A PHASE III STUDY OF PACRITINIB (PAC) VS BEST AVAILABLE THERAPY (BAT) IN PRIMARY MYELOFIBROSIS (PMF), POST- ... PERSIST-1: A PHASE III STUDY OF PACRITINIB (PAC) VS BEST AVAILABLE THERAPY (BAT) IN PRIMARY MYELOFIBROSIS (PMF), POST- ...
We present a case of a 36-year-old female patient with splenomegaly, underlying primary myelofibrosis, and detection of somatic ... Recurrent Upper Gastrointestinal Bleeding from Isolated Gastric Varices as Primary Symptom of Myelofibrosis: A Case Report on ... Endoscopic ultrasonography; Left-sided portal hypertension; Primary myelofibrosis; TKI; Therapeutic endoscopy; Variceal ... Recurrent Upper Gastrointestinal Bleeding from Isolated Gastric Varices as Primary Symptom ...
... has been used as palliative care for splenomegaly in patients with hematologic disorders such as primary myelofibrosis. [26] ... A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012 Mar 1. 366 (9):799-807. [QxMD ... recent developments in mitigating splenomegaly include the use of the JAK2 inhibitor ruxolitinib in primary myelofibrosis, ... Successful medical treatment of the primary disorder in cases of splenomegaly can lead to regression of the hypersplenism ...
  • These mutations are not specific to myelofibrosis, but are observed in other myeloproliferative neoplasms, specifically polycythemia vera and essential thrombocythemia. (wikipedia.org)
  • Primary myelofibrosis, also known as idiopathic myelofibrosis and agnogenic myeloid metaplasia, is a malignant disease, one of the chronic myeloproliferative neoplasms, along with polycythemia vera and essential thrombocythemia, amongst others (see table 1). (fcarreras.org)
  • Abstract In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity, distinct from essential thrombocythemia (ET). (pvreporter.com)
  • The 2016 revision of the World Health Organization (WHO) classification of myeloproliferative neoplasms defines 2 stages of primary myelofibrosis (PMF) that is, prefibrotic/early (pre-PMF) and overt fibrotic (overt-PMF) phase. (unipv.it)
  • The classic myeloproliferative neoplasms, including chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are a phenotypically diverse category of malignancies that are derived from stem cells in the myeloid lineage. (mhmedical.com)
  • Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (researchgate.net)
  • A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment). (medscape.com)
  • [ 5 ] Primary myelofibrosis is categorized as a chronic myeloproliferative disorder, along with chronic myelogenous leukemia (CML), polycythemia vera , and essential thrombocytosis . (medscape.com)
  • A proportion of patients with polycythemia vera and essential thrombocythemia will, over the course of time, develop symptoms of bone marrow fibrosis indistinguishable from that in primary myelofibrosis. (fcarreras.org)
  • The World Health Organization (WHO) classifies polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) as Ph-negative MPNs. (cancernetwork.com)
  • MF can present as primary myelofibrosis (PMF), or arise from a pre-existing diagnosis of polycythemia vera or essential thrombocythemia. (haematologica.org)
  • Post- polycythemia vera myelofibrosis. (cancer.gov)
  • the former constitutes the topic of this chapter and includes chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (mhmedical.com)
  • This is often found in essential thrombocythaemia, polycythemia vera and primary myelofibrosis. (edu.au)
  • Secondary myelofibrosis occurs when there is excessive red blood cell production (polycythemia vera) or excessive platelet production (essential thrombocythemia) that evolves into myelofibrosis. (fda.gov)
  • The approval of Inrebic for intermediate-2 or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis was based on the results of a clinical trial where 289 patients with myelofibrosis were randomized to receive two different doses (400 mg or 500 mg daily by mouth) of fedratinib or placebo. (fda.gov)
  • Subtypes include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (researchgate.net)
  • Total patients grew across myelofibrosis (MF), polycythemia vera (PV) and graft-versus-host disease (GVHD). (businesswire.com)
  • Mutations in the JAK2 , MPL , CALR , and TET2 genes are associated with most cases of primary myelofibrosis. (medlineplus.gov)
  • Individuals with refractory cases of primary myelofibrosis may require splenectomy or splenic irradiation. (medscape.com)
  • Mutations of the Janus kinase 2 ( JAK2 ) gene are present in a high proportion of cases of primary myelofibrosis. (msdmanuals.com)
  • However, there are rare cases of primary myelofibrosis in which none of these three mutations are present (triple negative primary myelofibrosis). (msdmanuals.com)
  • In 2016, prefibrotic primary myelofibrosis was formally classified as a distinct condition that progresses to overt PMF in many patients, the primary diagnostic difference being the grade of fibrosis. (wikipedia.org)
  • The primary feature of primary myelofibrosis is bone marrow fibrosis, but it is often accompanied by: Abdominal fullness related to an enlarged spleen (splenomegaly). (wikipedia.org)
  • 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)
  • The cause of the excessive marrow fibrosis observed in primary myelofibrosis remains unclear. (medscape.com)
  • Primary myelofibrosis (PMF) is a chronic myeloproliferative neoplasm characterized by bone marrow fibrosis, splenomegaly, and anemia with nucleated and teardrop-shaped red blood cells. (msdmanuals.com)
  • Malignant myelofibrosis (sometimes called acute myelofibrosis), is a rare variant of myelofibrosis characterized by pancytopenia, myeloblastosis, and marrow fibrosis that has a more rapidly progressive downhill course and is generally due to a type of acute leukemia called acute megakaryoblastic leukemia. (msdmanuals.com)
  • Bone marrow fibrosis leading to dry tap aspiration and often associated with blast crisis has previously been reported in both Chronic myeloid leukemia and Primary myelofibrosis. (peertechzpublications.org)
  • Here we present a case of Chronic myeloid leukemia in fibrosis and blast crisis in a 32 year old lady which closely resembled Primary myelofibrosis in transformation. (peertechzpublications.org)
  • Primary myelofibrosis (PMF) is a type of cancer of bone marrow and is characterized by bone marrow fibrosis, extramedullary hematopoiesis, and JAK2 tyrosine kinase mutations. (picmonic.com)
  • Reduced megakaryocytic expression of GATA1 has been linked to impaired hematopoiesis and bone marrow fibrosis in murine models and in vivo in patients affected by primary myelofibrosis (PMF). (ox.ac.uk)
  • Bone marrow fibrosis is a central pathological feature and World Health Organization major diagnostic criterion of myelofibrosis. (haematologica.org)
  • Although bone marrow fibrosis is seen in a variety of malignant and non-malignant disease states, the deposition of reticulin and collagen fibrosis in the bone marrow of patients with myelofibrosis is believed to be mediated by the myelofibrosis hematopoietic stem/progenitor cell, contributing to an impaired microenvironment favoring malignant over normal hematopoiesis. (haematologica.org)
  • However, modern myelofibrosis prognostication systems utilized in risk-adapted treatment approaches do not include bone marrow fibrosis as a prognostic variable. (haematologica.org)
  • The specific effect on bone marrow fibrosis of JAK2 inhibition, and other rationally based therapies currently being evaluated in myelofibrosis, has yet to be fully elucidated. (haematologica.org)
  • Hematopoietic stem cell transplantation remains the only curative therapeutic approach that reliably results in resolution of bone marrow fibrosis in patients with myelofibrosis. (haematologica.org)
  • Bone marrow scar formation (fibrosis) is a hallmark of myelofibrosis and contributes significantly to the disease progression. (lls.org)
  • There is an association between mutations to the JAK2, CALR, or MPL genes and myelofibrosis. (wikipedia.org)
  • 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)
  • These data led to the launch of a double-blind, randomized phase 3 trial examining the safety of luspatercept compared with placebo in patients with myeloproliferative neoplasm-associated myelofibrosis who are on concomitant JAK2 inhibitor therapy and require red blood cell transfusions. (onclive.com)
  • Post- essential thrombocythemia myelofibrosis. (cancer.gov)
  • [ 20 ] The JAK inhibitor fedratinib is approved treatment of adults with intermediate-2 or high-risk primary or secondary (post-PV or post-essential thrombocythemia) myelofibrosis. (medscape.com)
  • Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. (ac.rs)
  • TY - JOUR AU - Leković, Danijela AU - Bogdanović, Andrija AU - Sobas, Marta AU - Arsenović, Isidora AU - Smiljanić, Mihailo AU - Ivanović, Jelena AU - Bodrožić, Jelena AU - Čokić, Vladan AU - Milić, Nataša PY - 2023 UR - http://rimi.imi.bg.ac.rs/handle/123456789/1344 AB - Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. (ac.rs)
  • To determine whether umbilical cord blood transplantation (UCBT) is an alternative cure for myelofibrosis (MF), we evaluated 35 UCBTs reported to Eurocord. (nih.gov)
  • An allogenic transplant is, at the present time, the only treatment that offers the possibility of a cure for myelofibrosis. (fcarreras.org)
  • Primary myelofibrosis treatment options include stem cell transplantation, transfusion, and ruxolitinib. (picmonic.com)
  • In this article, we provide updated data on ruxolitinib therapy for patients with myelofibrosis and offer expert opinion on the appropriate use of this agent in the community practice. (cancernetwork.com)
  • To date, 2 JAK inhibitors have received approval for use in patients with myelofibrosis: ruxolitinib and fedratinib. (onclive.com)
  • In the phase 2 MANIFEST trial (NCT02158858), investigators are examining CPI-0610 in combination with ruxolitinib in patients with myelofibrosis who had not received prior treatment with JAK inhibitors (Arm 3). (onclive.com)
  • The Phase 3 LIMBER-304 trial, evaluating parsaclisib in combination with ruxolitinib BID in suboptimal responders in MF and the Phase 3 LIMBER-313 trial, evaluating parsaclisib in combination with ruxolitinib BID in first-line MF, were discontinued following results of interim analyses that indicated that the studies were unlikely to meet their primary endpoints in the intent-to-treat patient population. (businesswire.com)
  • Portal hypertension occurs in approximately 7% of patients with primary myelofibrosis and may be related to increased portal flow resulting from marked splenomegaly and to intrahepatic obstruction resulting from thrombotic obliteration of small portal veins. (medscape.com)
  • We present a case of a 36-year-old female patient with splenomegaly , underlying primary myelofibrosis , and detection of somatic Janus- kinase -2 driver- mutation JAK2V617F. (bvsalud.org)
  • Successful medical treatment of the primary disorder in cases of splenomegaly can lead to regression of the hypersplenism without the need for surgery. (medscape.com)
  • Low-dose radiotherapy has been used as palliative care for splenomegaly in patients with hematologic disorders such as primary myelofibrosis. (medscape.com)
  • It is beneficial in patients with intermediate or high-risk disease based on the prognostic scoring system from the International Working Group for Myelofibrosis Research and Treatment. (picmonic.com)
  • This prognostic scoring system for primary myelofibrosis resulted from data from 1054 consecutively diagnosed patients with PMF from 1980 to 2007. (medscape.com)
  • New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. (medscape.com)
  • Patients with myelofibrosis have an increased risk of acute meyloid leukemia and frank bone marrow failure. (wikipedia.org)
  • Patients with primary myelofibrosis are also prone to developing infectious complications because of defects in humoral immunity. (medscape.com)
  • In patients with primary myelofibrosis, the hematopoietic system is most affected. (medscape.com)
  • In both cases patients need to be monitored regularly at a centre with experience of dealing with myelofibrosis. (fcarreras.org)
  • However, given that it carries a high risk of mortality and morbidity, this procedure is reserved for young patients, in an otherwise generally good state of health, but who have been diagnosed with a myelofibrosis with a poor prognosis. (fcarreras.org)
  • In many patients, myelofibrosis is asymptomatic. (msdmanuals.com)
  • Purpose To develop a prognostic system for transplantation-age patients with primary myelofibrosis (PMF) that integrates clinical, cytogenetic, and mutation data. (unimi.it)
  • Treatment of primary myelofibrosis in patients without symptoms is usually watchful waiting. (mnoncology.com)
  • Peripheral blood smear of patients with primary myelofibrosis can show dacrocytes, which results from red blood cells squeezing out from fibrotic tissue on bone marrow. (picmonic.com)
  • The myelofibrosis paradigm is bursting with novel agents and combinations that have been developed with the ultimate goal of helping patients live longer. (onclive.com)
  • The myelofibrosis paradigm is bursting with novel agents and combinations that have been developed with the ultimate goal of helping patients live longer, said Srdan Verstovsek, MD, PhD, who added that several phase 3 trial are underway to bring more options into the clinic. (onclive.com)
  • The National Comprehensive Cancer Network guidelines for the treatment of patients with myelofibrosis are based on risk of death, as well as symptoms and signs, said Verstovsek. (onclive.com)
  • Luspatercept-aamt (Reblozyl), which received FDA approval in April 2020 for myelodysplastic syndromes-associated anemia, is now under exploration in patients with myelofibrosis-associated anemia in a phase 2 trial. (onclive.com)
  • Today, the U.S. Food and Drug Administration approved Inrebic (fedratinib) capsules to treat adult patients with certain types of myelofibrosis. (fda.gov)
  • Prior to today, there was one FDA-approved drug to treat patients with myelofibrosis, a rare bone marrow disorder. (fda.gov)
  • As a result of treatment with Inrebic, 36 patients experienced greater than or equal to a 50% reduction in myelofibrosis-related symptoms, such as night sweats, itching, abdominal discomfort, feeling full sooner than normal, pain under ribs on left side, and bone or muscle pain. (fda.gov)
  • Patients with related conditions, essential thromboycytosis (ET) and primary myelofibrosis, also can carry the mutation. (cdc.gov)
  • The same 5 variables established above had significant impact on survival when analyzed as time-dependent covariates in 525 primary MF patients. (medscape.com)
  • Watch this on-demand webcast from an ASH satellite symposium on the use of current and emerging therapies for patients with myelofibrosis, including discussions on individualized treatment plans, treatment for relapsed/refractory disease, managing treatment-related toxicities, and clinical trials. (clinicaloptions.com)
  • This program is intended for physicians and other healthcare professionals who care for patients with myelofibrosis, including hematologists/oncologists, advanced care practitioners, pharmacists, and nurses. (clinicaloptions.com)
  • 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)
  • Other common signs and symptoms of primary myelofibrosis include fever, night sweats, and bone pain. (medlineplus.gov)
  • Do my symptoms mean I have myelofibrosis? (healthgrades.com)
  • Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. (wikipedia.org)
  • Diagnosis requires bone marrow aspirate and biopsy and exclusion of other conditions that can cause myelofibrosis (secondary myelofibrosis). (msdmanuals.com)
  • Mutational spectrum defines primary and secondary myelofibrosis. (llni.co.uk)
  • We have a single study at the dose and formulation that's been proposed, and it unfortunately didn't achieve evidence of efficacy for either the primary or secondary endpoints," said committee member Caleb Alexander, MD, a professor of epidemiology and medicine at Johns Hopkins University, Baltimore, Maryland, who voted against approval. (medscape.com)
  • 1] PV and ET are both capable of progressing to a fibrotic stage that clinically resembles PMF, and collectively these three disease entities are termed myelofibrosis (MF). (cancernetwork.com)
  • The DIPSS Prognosis in Myelofibrosis calculator is created by QxMD. (medscape.com)
  • However, in primary myelofibrosis, the excess collagen forms scar tissue in the bone marrow. (medlineplus.gov)
  • Myelofibrosis is a reactive, reversible increase in bone marrow collagen often with extramedullary hematopoiesis (primarily in the spleen). (msdmanuals.com)
  • These primary myelofibrosis progeny cells stimulate bone marrow fibroblasts (which are not part of the neoplastic transformation) to secrete excessive collagen. (msdmanuals.com)
  • Myelofibrosis (MF) is a hematopoietic stem cell malignancy classified as a myeloproliferative neoplasm (MPN). (cancernetwork.com)
  • 3 Myelofibrosis (MF) refers to the Philadelphia chromosome ( BCR-ABL1 )-negative myeloproliferative neoplasm (MPN) originating at the level of the multipotent hematopoietic stem cell. (haematologica.org)
  • Primary myelofibrosis is characterised by the presence of a fibrous tissue in the bone marrow, and by the migration of bone marrow stem cells to the blood, where they colonise organs from a distance (mainly the spleen and the liver). (fcarreras.org)
  • Primary myelofibrosis is a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells. (medscape.com)
  • Primary myelofibrosis results from neoplastic transformation of a multipotent bone marrow stem cell. (msdmanuals.com)
  • the mutation causing this variant is found in approximately half of individuals with primary myelofibrosis. (wikipedia.org)
  • Some people with primary myelofibrosis do not have a mutation in any of the known genes associated with this condition. (medlineplus.gov)
  • Paradoxically, loss of Nol3 also leads to hematological disruption in mice resulting in a myeloproliferative neoplasm resembling primary myelofibrosis (PMF), and its deletion has also been detected in patient samples of PMF. (wikipedia.org)
  • The MPN designation also includes essential thrombocytosis (ET) and primary myelofibrosis (PMF). (cdc.gov)
  • v Naymagon L, Mascarenhas J. Myelofibrosis-Related Anemia: Current and Emerging Therapeutic Strategies. (gsk.com)
  • Prognostic relevance of anemia and transfusion dependency in myelodysplastic syndromes and primary myelofibrosis. (gsk.com)
  • Myelofibrosis is a clonal neoplastic disorder of hematopoiesis, the formation of blood cellular components. (wikipedia.org)
  • Myelofibrosis is a chronic disorder where scar tissue forms in the bone marrow and the production of the blood cells moves from the bone marrow to the spleen and liver, causing organ enlargement. (fda.gov)
  • Allogeneic stem cell transplantation is a potential treatment for primary myelofibrosis. (picmonic.com)
  • This project explores the connection between the niche - the area in the bone marrow where blood cells are formed - and the development of leukemia stem cells (LSCs) that give way to primary myelofibrosis (PMF). (lls.org)
  • Extramedullary hematopoiesis in the spleen of a patient with primary myelofibrosis. (medscape.com)
  • The spleen works as the primary site of extramedullary hematopoiesis. (picmonic.com)
  • Myelofibrosis (MF) is a serious and rare blood cancer affecting nearly 25,000 people in the U.S. i It involves the buildup of excessive scar tissue in the bone marrow, which interferes with the production of healthy blood cells. (gsk.com)
  • Primary myelofibrosis: this condition causes scar tissue to grow inside the bone marrow. (massgeneral.org)
  • 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)
  • Mutations of the thrombopoietin receptor gene ( MPL ) or the calreticulin ( CALR ) gene also may be the cause of primary myelofibrosis. (msdmanuals.com)
  • In primary myelofibrosis, nucleated red blood cells (normoblasts) and myelocytes are released into the circulation (leukoerythroblastosis) when there is extramedullary hematopoiesis (ie, non-marrow organs have taken over blood cell production because of the fibrosed marrow). (msdmanuals.com)
  • Extramedullary hematopoiesis occurs over the course of primary myelofibrosis. (picmonic.com)
  • We use mouse genetics to model myelofibrosis and understand the cellular and molecular makeup of the diseased microenvironment. (lls.org)
  • We aim to understand the composition and alteration of the bone marrow microenvironment in myelofibrosis. (lls.org)
  • Myelofibrosis is a very heterogeneous disease at the clinical level and it requires individualised treatment that is adjusted to risk. (fcarreras.org)
  • viii Bose P., Verstovsek S. JAK Inhibition for the Treatment of Myelofibrosis: Limitations and Future Perspectives. (gsk.com)
  • approval was based on the results of the COMFORT (COntrolled MyeloFibrosis study with ORal JAK inhibitor Treatment) I and II studies. (cancernetwork.com)
  • Recurrent Upper Gastrointestinal Bleeding from Isolated Gastric Varices as Primary Symptom of Myelofibrosis: A Case Report on Combining Interventional and Pharmacologic Treatment Options. (bvsalud.org)
  • Primary purpose: Treatment. (who.int)
  • When myelofibrosis occurs on its own, it is called primary myelofibrosis. (fda.gov)