• Fraticelli P, Kafyeke A, Mattioli M, Martino GP, Murri M, Gabrielli A. Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib. (wjgnet.com)
  • Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm 3 , a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. (wjgnet.com)
  • PDGFRA -associated chronic eosinophilic leukemia is often grouped with a related condition called hypereosinophilic syndrome. (medlineplus.gov)
  • Bain BJ: Eosinophilic leukemias and the idiopathic hypereosinophilic syndrome. (karger.com)
  • Besides genetics, ICC for the first time has introduced bone marrow morphologic criteria in distinguishing idiopathic hypereosinophilia/hypereosinophilic syndrome from chronic eosinophilic leukemia, not otherwise specified. (bvsalud.org)
  • Hypereosinophilic syndrome was previously considered to be idiopathic, but molecular characterization has revealed that many cases have specific clonal disorders. (msdmanuals.com)
  • A thorough understanding of the idiopathic hypereosinophilic syndrome (IHES) and further optimization of diagnostic work-up procedures are warranted. (oncotarget.com)
  • Differential diagnoses include drug allergies and parasitic infections, solid and hematological malignancies (i.e. chronic myeloid leukemia), eosinophilic granulomatosis with polyangiitis and human T cell lymphotropic virus infection (see these terms). (orpha.net)
  • May provide an important clue for less common difficult-to-diagnose conditions, such as eosinophilic granulomatosis with polyangiitis, IgG4-related disease, and Rosai-Dorfman disease. (thebloodproject.com)
  • Occasionally, people with PDGFRA -associated chronic eosinophilic leukemia develop other blood cell cancers, such as acute myeloid leukemia or B-cell or T-cell acute lymphoblastic leukemia or lymphoblastic lymphoma. (medlineplus.gov)
  • Occasionally, the FIP1L1-PDGFRA fusion can be identified in patients with acute myeloid leukemia or B-cell or T-cell acute lymphoblastic leukemia or lymphoblastic lymphoma and sporadically in myeloid sarcoma (Metzgeroth et al. (atlasgeneticsoncology.org)
  • Hypereosinophilia (i.e. blood eosinophil counts at or above 1,500 per microliter) or, less commonly, eosinophilia (counts above 500 but below 1,500 per microliter) are found in the vast majority of cases of eosinophilic myocarditis and are valuable clues that point to this rather than other types of myocarditis or myocardial injuries. (wikipedia.org)
  • There are many causes of eosinophilia that may underlie eosinophilic myocarditis. (wikipedia.org)
  • Primary eosinophilia is not a reactive phenomenon and can be described as either clonal or idiopathic in nature. (medscape.com)
  • If reactive causes are ruled out and no underlying clonal origin is proven, the eosinophilia is described as idiopathic. (medscape.com)
  • An interstitial deletion del(4)(q12q12) generating a FIP1L1-PDGFRA fusion gene is observed in diverse eosinophilia-associated hematologic disorders like hyperseosinophilic syndrome (HES), systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL). (atlasgeneticsoncology.org)
  • Marked and sustained eosinophilia eventually leads to eosinophilic infiltration and functional damage of peripheral organs, most commonly the heart, skin, lungs, or nervous system. (atlasgeneticsoncology.org)
  • In this article, the term eosinophilia is defined as an increase in peripheral blood eosinophilic leukocytes to more than 600 cells per microliter (μL) of blood. (medscape.com)
  • We analyzed purified eosinophils from patients with IHES by next-generation whole-exome sequencing and compared DNA methylation profiles from reactive eosinophilic conditions to known clonal and suspected clonal eosinophilia. (oncotarget.com)
  • In healthy individuals, eosinophilic granulocytes (eosinophils) constitute less than five percent of all white blood cells [ 1 ], and in clinical practice blood eosinophilia is defined as an eosinophil count ≥ 0.5x10 9 /l. (oncotarget.com)
  • Eosinophilic myocarditis is inflammation in the heart muscle that is caused by the infiltration and destructive activity of a type of white blood cell, the eosinophil. (wikipedia.org)
  • This rarest form of the disorder reflects a rapidly progressive and extensive eosinophilic infiltration of the heart that is accompanied by massive myocardial cell necrosis. (wikipedia.org)
  • 1.5 109/L) and /or eosinophilic infiltration of tissue(s) responsible for target-organ damage. (orpha.net)
  • Interestingly, the T674I mutation that is analogous to the T315I mutation of BCR-ABL1 in chronic myeloid leukemia also confers imatinib resistance (Cools et al. (atlasgeneticsoncology.org)
  • Acute Myeloid Leukemia (AML) In acute myeloid leukemia (AML), malignant transformation and uncontrolled proliferation of an abnormally differentiated, long-lived myeloid progenitor cell results in high circulating numbers. (msdmanuals.com)
  • It is a secondary change associated with such disorders as inflammation, bone marrow necrosis, bone marrow injury, and disorders of myeloproliferation (e.g., acute myeloid leukemia) and lymphoproliferation (e.g., lymphoma). (nih.gov)
  • Dasatinib (Sprycel): Indicated for the treatment of adult patients with chronic myeloid leukemia in chronic, accelerated, or myeloid or lymphoid blast phase who are resistant or intolerant to prior therapy including imatinib. (medscape.com)
  • Data support a FIP1L1-PDGFRA fusion incidence of approximately 10-20% among patients presenting with idiopathic hypereosinophilia (Gotlib and Cools, 2008). (atlasgeneticsoncology.org)
  • Acquired (autoimmune) hemolytic anemia, congenital (erythroid) hypoplastic anemia (Diamond-Blackfan anemia), idiopathic thrombocytopenic purpura in adults and selected cases of secondary thrombocytopenia. (com.bd)
  • Leukemias and lymphomas. (com.bd)
  • The specific treatment (i.e. treatment other than measures to support the cardiovascular system) of eosinophilic myocarditis differs from the specific treatment of other forms of myocarditis in that it is focused on relieving the underlying reason for the excessively high numbers and hyperactivity of eosinophils as well as on inhibiting the pathological actions of these cells. (wikipedia.org)
  • These causes are classified as primary (i.e. a defect intrinsic to the eosinophil cell line), secondary (induced by an underlying disorder that stimulates the proliferation and activation of eosinophils), or idiopathic (i.e. unknown cause). (wikipedia.org)
  • However, these circumstances do not account for the increased number of eosinophils in PDGFRA -associated chronic eosinophilic leukemia. (medlineplus.gov)
  • Another characteristic feature of PDGFRA -associated chronic eosinophilic leukemia is organ damage caused by the excess eosinophils. (medlineplus.gov)
  • When the FIP1L1-PDGFRA fusion gene mutation or point mutations in the PDGFRA gene occur in blood cell precursors, the growth of eosinophils (and occasionally other blood cells, such as neutrophils and mast cells) is poorly controlled, leading to PDGFRA -associated chronic eosinophilic leukemia. (medlineplus.gov)
  • PDGFRA-associated chronic eosinophilic leukemia is a form of blood cell cancer characterized by an elevated number of cells called eosinophils in the blood. (nih.gov)
  • 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)
  • The World Health Organization (WHO) provides diagnostic criteria for BCR-ABL negative MPNs, including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), chronic eosinophilic leukemia (CEL), chronic neutrophilic leukemia (CNL), and myeloproliferative neoplasms, unclassifiable (MPN-U) (1). (iranpath.org)
  • Myeloproliferative diseases are a heterogeneous group of disorders characterized by cellular proliferation of one or more hematologic cell lines in the peripheral blood, distinct from acute leukemia. (medscape.com)
  • There are 6 types of chronic myeloproliferative neoplasms. (cancerhealth.com)
  • Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myeloproliferative neoplasms. (cancerhealth.com)
  • Chronic myeloproliferative neoplasms sometimes become acute leukemia , in which too many abnormal white blood cells are made. (cancerhealth.com)
  • Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by the overproduction of RBCs. (lecturio.com)
  • Bone marrow fibrosis is also detected in the spent phase of chronic myelogenous leukemia and polycythemia vera. (medscape.com)
  • In large cell lymphoma and leukemia cells involvement of body fluid this concept becomes less challenging. (cytojournal.com)
  • Large cell lymphoma and leukemia cells tend to have large size nuclei, less mature chromatin, and visible nucleoli with and without cytoplasmic vacuoles. (cytojournal.com)
  • This department provides medical care for patients with blood disorders, including anemia and thrombocytopenia, and neoplastic disorders such as leukemia and malignant lymphoma. (japanhospitalsearch.org)
  • In addition, he works closely with investigators in the Bloomfield Prognostic Center, Alliance for Clinical Trials in Oncology, and Leukemia and Lymphoma Society Beat AML group to develop biomarkers that predict response to targeted therapy in leukemia. (uc.edu)
  • The most common genetic abnormality in PDGFRA -associated chronic eosinophilic leukemia results from a deletion of genetic material from chromosome 4 , which brings together part of the PDGFRA gene and part of the FIP1L1 gene, creating the FIP1L1-PDGFRA fusion gene. (medlineplus.gov)
  • however chronic eosinophilic leukemia with FIP1L1-PDGFRA is likely to be responsive also to dasatinib, nilotinib, sorafenib and midostaurin (PKC412) (Lierman et al. (atlasgeneticsoncology.org)
  • Perhaps less commonly, eosinophilic myocarditis, eosinophilic thrombotic myocarditis, and eosinophilic fibrotic myocarditis are viewed as three separate but sequentially linked disorders in a spectrum of disorders termed eosinophilic cardiac diseases. (wikipedia.org)
  • Increased levels of eosinophilic leukocytes in the blood can be idiopathic, or may result from a variety of conditions, including connective tissue diseases, helminthic infections, neoplasias, and allergic disorders. (medscape.com)
  • however the incidence rates for molecularly defined eosinophilic disorders are not known. (atlasgeneticsoncology.org)
  • Based on new data and increased understanding of disease molecular genetics, the international consensus classification (ICC) has made several changes in the diagnosis and classification of eosinophilic disorders and systemic mastocytosis. (bvsalud.org)
  • Is most commonly associated with liver disease, acute or chronic inflammation, autoimmune disorders and certain malignancies. (thebloodproject.com)
  • It is distinguished from non-eosinophilic myocarditis, which is heart inflammation caused by other types of white blood cells, i.e. lymphocytes and monocytes, as well as the respective descendants of these cells, NK cells and macrophages. (wikipedia.org)
  • This distinction is important because the eosinophil-based disorder is due to a particular set of underlying diseases and its preferred treatments differ from those for non-eosinophilic myocarditis. (wikipedia.org)
  • Eosinophilic myocarditis is often viewed as a disorder that has three progressive stages. (wikipedia.org)
  • The focus here is on eosinophilic myocarditis as a distinct disorder separate from its thrombotic and fibrotic sequelae. (wikipedia.org)
  • Eosinophilic myocarditis is a rare disorder. (wikipedia.org)
  • Symptoms in eosinophilic myocarditis are highly variable. (wikipedia.org)
  • Cardiac manifestations of eosinophilic myocarditis range from none to life-threatening conditions such as cardiogenic shock or sudden death due to abnormal heart rhythms. (wikipedia.org)
  • In its most extreme form, however, eosinophilic myocarditis can present as acute necrotizing eosinophilic myocarditis, i.e. with symptoms of chaotic and potentially lethal heart failure and heart arrhythmias. (wikipedia.org)
  • Other, less specific laboratory findings implicate a cardiac disorder but not necessarily eosinophilic myocarditis. (wikipedia.org)
  • The eosinophilic proliferation was severe in peripheral blood and bone marrow, and they revealed marked dysplastic features. (karger.com)
  • Eosinophilic differentiation occurs in the bone marrow from myeloid progenitors through the actions of GM-CSF, IL-3, and IL-5. (medscape.com)
  • Chronic myelogenous leukemia is a disease in which too many white blood cells are made in the bone marrow . (cancerhealth.com)
  • Equivalent alveolar proteinaceous materials may be seen in several inhalational exposures including silica, aluminum P85B or titanium, and can be observed in pneumocystis pneumonia also, pulmonary leukemia and nocardiosis. (idplink.net)
  • Berylliosis, fulminating or disseminated pulmonary tuberculosis when used concurrently with appropriate antituberculous chemotherapy, idiopathic eosinophilic pneumonias, symptomatic sarcoidosis. (com.bd)
  • As a result, the role of chronic pulmonary inflammation in the pathophysiology of asthma has been studied extensively in human and in animal models. (biomedcentral.com)
  • This department provides medical care for patients with acute and chronic kidney diseases, kidney failure, dysfunction and tumors of endocrine organs, and hypertension. (japanhospitalsearch.org)
  • Whole-exome sequencing and genome-wide methylation analyses identify novel disease associated mutations and methylation patterns in idiopathic. (oncotarget.com)
  • Lung disease/chronic hypoxia Hypoxia Sub-optimal oxygen levels in the ambient air of living organisms. (lecturio.com)
  • 2010. Chronic inflammation and secondary myelofibrosis. (nih.gov)
  • 4 Multiple etiologies may exist that, either alone or combined, create the inflammation.4 Possible causative agents include an inflammatory response to plaque, viruses (particularly upper respiratory), Bartonella henselae infection, or altered immune status (feline immunodeficiency or leukemia viruses, FIV or FeLV). (todaysveterinarypractice.com)
  • See the PDQ summary on Chronic Myelogenous Leukemia Treatment for information on diagnosis , staging , and treatment. (cancerhealth.com)
  • 600 cells/µL) indicates an eosinophilic disorder. (medscape.com)
  • They are also at risk of developing secondary acute leukemia from their underlying disorder, as well as from their treatment. (medscape.com)
  • Hematopoietic stem cell transplantation can be considered in young patients with chronic myelogenous leukemia in chronic phase if a human leukocyte antigen (HLA)-matched donor is available. (medscape.com)
  • Fibrosis (secondary) should be diagnosed and graded in subchronic and chronic studies only when it is a prominent feature of a lesion. (nih.gov)
  • To induce a diuresis or remission of proteinuria in idiopathic nephrotic syndrome or that due to lupus erythematosus. (com.bd)
  • In humans, myelofibrosis is an abbreviated term that has been used in reference to chronic idiopathic myelofibrosis or primary myelofibrosis. (nih.gov)
  • Collagen fibers are identified as eosinophilic fibers that may be in bundles and are confirmed with the use of a trichrome stain (e.g. (nih.gov)
  • People with PDGFRA -associated chronic eosinophilic leukemia can also have an enlarged spleen (splenomegaly) and elevated levels of certain chemicals called vitamin B12 and tryptase in the blood. (medlineplus.gov)
  • In chronic conditions requiring long-term therapy the lowest dosage that provides adequate, but not necessarily complete, relief should be used. (com.bd)
  • Cushing's syndrome, and Hyperglycemia: Monitor patients for these conditions with chronic use. (recallguide.org)
  • In addition, the WBC and platelet counts are also increased, which differentiates PV from erythrocytosis seen with chronic hypoxia and other chronic conditions. (lecturio.com)
  • Effects of environmental exposures on the programming of biological mechanisms related to chronic conditions in childhood including respiratory and neurodevelopment/mental health outcomes. (uc.edu)
  • Pathological differentiation of PAP from these various other entities needs cytospin analysis, which reveals regular acid-Schiff-positive generally, GMS stain-negative, eosinophilic, granular, acellular materials using a few enlarged foamy macrophages (1). (idplink.net)