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precursors b. Fibrotic phase - collagenous fibrosis with lack of marrow
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What Are Myeloproliferative Disorders?


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6. ↑ Levine RL, Wadleigh M, Cools J, et al. (2005). "Activating mutation in the tyrosine kinase JAK2 in
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50 Myeloproliferative Disease

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50 Myeloproliferative Disease

  1. 1. Web Εικόνες Περισσότερα MSN Hotmail Είσοδος | Ελλάδα | Προτιμήσεις Bing myeloproliferative disorders Beta ΌΛΑ ΤΑ REFERENCE » WIKIPEDIA ARTICLES ΑΠΟΤΕΛΈΣΜΑΤΑ Αναφορά Myeloproliferative disease view original wikipedia article Myeloproliferative disease The myeloproliferative diseases ("MPD"s) Myeloproliferative disease are a group of diseases of the bone Classification and external resources marrow in which excess cells are ICD-10 D47.1 overview outline images locations produced. They are related to, and may ICD-9 205.1, 238.4, 289.89, 289.9 evolve into, myelodysplastic syndrome and Search this article ICD-O: 9950/0-9964/3 high acute myeloid leukemia, although the MeSH D009196 myeloproliferative diseases on the whole have a much better prognosis than these conditions. The concept of myeloproliferative Myeloproliferative disease disease was first proposed in 1951 by the eminent hematologist William Dameshek.[1] Classification In the most recent World Health Organization classification of Hematologic Causes malignancies, this group of diseases was renamed from "myeloproliferative diseases" to Diagnosis "myeloproliferative neoplasms". This reflects the underlying clonal genetic changes that References are a salient feature of this group of disease. External links Images Videos Classification Although not a malignant neoplasm like other cancers, MPDs are classified within the hematological neoplasms. There are four main myeloproliferative diseases, which can be further categorized by the view all 24 view all 15 presence of the Philadelphia chromosome: Philadelphia Chromosome "positive" Philadelphia Chromosome "negative" Chronic myelogenous leukemia (CML) Polycythemia vera (PV) Essential thrombocytosis (ET) Myelofibrosis (MF) In 2001, the World Health Organization classified "chronic eosinophilic leukemia / hypereosinophilic syndrome" and chronic neutrophilic leukemia under "Chronic myeloproliferative diseases". [2] Causes All MPDs arise from precursors of the "myeloid" lineage in the bone marrow. The lymphoid lineage may produce similar diseases, the lymphoproliferative disorders (acute lymphoblastic leukemia, lymphomas, chronic lymphocytic leukemia and multiple myeloma). Diagnosis Depending on the nature of the myeloproliferative disorder, diagnostic tests may include red cell mass determination (for polycythemia), bone marrow aspirate and trephine biopsy, arterial oxygen saturation and carboxyhaemoglobin level, neutrophil alkaline phosphatase level, vitamin B12 (or B12 binding capacity) and serum urate. [3] According to the WHO Classification of Hematopoietic and Lymphoid Neoplasms 2008 myeloproliferative disorders are divided into the following by diagnostic characteristics: 1. Chronic myelogenous leukemia (CML) with defining translocation t(9;22) BCR-ABL translocation which has three breakpoints: a. u-BCR-ABL (p230): leads to CML with usual neutrophilia and basophilia. b. minor-BCR-ABL (p190): leads to CML which has a tendency to become acute lymphoblastic leukemia (ALL) usually precursor B ALL and rarely precursor T ALL. c. major-BCR-ABL (p210): normal usual breakpoint 2. Primary myelofibrosis associated with JAK2 mutation in up to 50% of cases and MPL (thrombopoietin receptor) mutation in up to 5% of cases a. Cellular phase - increased megakaryocytes which cluster, reticulin fibrosis, later trichrome (collagenous) fibrosis, and increased myeloid
  2. 2. precursors b. Fibrotic phase - collagenous fibrosis with lack of marrow elements 3. Polycythemia vera associated most often with JAK2 mutation in up to 80% of cases a. Cellular phase - increased megakaryocytes which cluster, reticulin fibrosis, later trichrome fibrosis, and increased myeloid and erythroid precursors b. Fibrotic phase - collagenous fibrosis with lack of marrow elements 4. Essential Thrombocythemia associated with JAK2 mutation in up to 20% of cases and MPL (thrombopoietin receptor) mutation in up to 15% of cases a. Cellular phase - increased large megakaryocytes with fibrosis and little increase in other bone marrow elements b. Fibrotic phase - collagenous fibrosis with lack of marrow elements These disorders are still being revised according to more specific genetic mutations and how often patients end in a fibrotic marrow event. In 2005, the discovery of the JAK2 V617F mutation provided some evidence to suggest a common pathogenesis for the Philadelphia Chromosome negative MPDs.[4][5][6][7][8] References 1. ↑ Dameshek W (1951). "[Expression error: Missing operand for > Some speculations on the myeloproliferative syndromes]". Blood 6 (4): 372–5. PMID 14820991. 2. ↑ "Classification of Human Hematopoietic Malignancies". http://emice.nci.nih.gov/emice/mouse_models/organ_models/hema_models/hema_human_class. 3. ↑ Levene, Malcolm I.; Lewis, S. M.; Bain, Barbara J.; Imelda Bates (2001). Dacie & Lewis Practical Haematology. London: W B Saunders. p. 586. ISBN 0-443-06377-X. 4. ↑ Baxter EJ, Scott LM, Campbell PJ, et al. (2005). "[Expression error: Missing operand for > Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders]". Lancet 365 (9464): 1054– 1061. doi:10.1016/S0140-6736(05)71142-9. PMID 15781101. 5. ↑ James C, Ugo V, Le Couedic JP, et al. (2005). "[Expression error: Missing operand for > A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera]". Nature 434 (7037): 1144–1148. doi:10.1038/nature03546. PMID 15793561. 6. ↑ Levine RL, Wadleigh M, Cools J, et al. (2005). "[Expression error: Missing operand for > Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis]". Cancer Cell 7 (4): 387–397. doi:10.1016/j.ccr.2005.03.023. PMID 15837627. 7. ↑ Kralovics R, Passamonti F, Buser AS, et al. (2005). "[Expression error: Missing operand for > A gain- of-function mutation of JAK2 in myeloproliferative disorders]". N Engl J Med 352 (17): 1779–1790. doi:10.1056/NEJMoa051113. PMID 15858187. 8. ↑ Campbell PJ, Scott LM, Buck G, et al. (2005). "[Expression error: Missing operand for > Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study]". Lancet 366 (9501): 1945–1953. doi:10.1016/S0140-6736(05)67785- 9. PMID 16325696. External links Myeloproliferative Disorders Website of The CMPD Education Foundation Myeloproliferative Disorders in practice Myeloproliferative Disease Support List Free daily digest, 2850 subscribers 40+ countries MeSH Myeloproliferative+Disorders Myeloproliferative Disorders Research Consortium MPD Foundation - Free newsletter and patient brochure This disease article is a stub. You can help Wikipedia by expanding it. Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, show 200-208) Categories: Disease stubs | Hematology History View article history All Wikipedia content is licensed under the GNU Free Document License or the Creative Commons CC-BY-SA license or is otherwise used here in compliance with the Copyright Act Go to Bing in English © 2010 Microsoft | Προστασία προσωπικών δεδομένων | Νομικές ανακοινώσεις | Βοήθεια
  3. 3. In English | En español What Are Myeloproliferative Disorders? Quick Links Myeloproliferative disorders are a group of slow-growing blood cancers, including chronic myelogenous leukemia, in which large numbers of abnormal red blood cells, white blood cells, or platelets grow and Director's Corner spread in the bone marrow and the peripheral blood. Dictionary of Cancer Terms NCI Drug Dictionary The following PDQ treatment summaries are available: Funding Opportunities Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies NCI Publications Includes childhood myelodysplastic syndromes and other myeloproliferative disorders. Advisory Boards and Groups [ patients ] [ health professionals ] Science Serving People Español Acute Myeloid Leukemia [ patients ] [ health professionals ] Questions about cancer? Chronic Myelogenous Leukemia 1-800-4-CANCER [ patients ] [ health professionals ] Chronic Myeloproliferative Disorders [ patients ] [ health professionals ] Subsections of this summary include: NCI Highlights Polycythemia Vera Office of Biorepositories and Chronic Idiopathic Myelofibrosis Biospecimen Research Essential Thrombocythemia The Nation's Investment in Chronic Neutrophilic Leukemia Cancer Research FY 2010 Chronic Eosinophilic Leukemia Report to the Nation Finds Myelodysplastic Syndromes Continued Declines in Cancer Rates Includes refractory anemia, refractory anemia with excess blasts, refractory anemia with ringed sideroblasts, refractory cytopenia with multilineage dysplasia, unclassifiable myelodysplastic syndrome, and myelodysplastic syndrome associated with del (5q). [ patients ] [ health professionals ] Subsections of this summary include: De novo Myelodysplastic Syndrome Secondary Myelodysplastic Syndrome Previously Treated Myelodysplastic Syndrome Myelodysplastic/Myeloproliferative Diseases [ patients ] [ health professionals ] Subsections of this summary include: Chronic Myelomonocytic Leukemia Juvenile Myelomonocytic Leukemia Atypical Chronic Myeloid Leukemia Myelodysplastic/Myeloproliferative Disease, Unclassifiable Back to Top NCI Home | Text-Only Version | Contact Us | Policies | Accessibility | RSS | Viewing Files | FOIA | Site Help | Site Map
  4. 4. A Service of the National Cancer Institute
  5. 5. Web Εικόνες Περισσότερα MSN Hotmail Είσοδος | Ελλάδα | Προτιμήσεις Bing essential thrombocythemia Beta ΌΛΑ ΤΑ REFERENCE » WIKIPEDIA ARTICLES ΑΠΟΤΕΛΈΣΜΑΤΑ Αναφορά Essential thrombocytosis view original wikipedia article Essential thrombocytosis 'Essential thrombocytosis (ET, also known ICD10 = D75.2, D47.3 as essential thrombocythemia) is a rare Classification and external resources chronic blood disorder characterized by ICD-9 238.71 overview outline images locations the overproduction of platelets by ICD-O: M9962/3 megakaryocytes in the bone marrow in Search this article OMIM 187950 high the absence of an alternative cause. In DiseasesDB 4522 some cases this disorder may be MedlinePlus 000543 progressive, and rarely may evolve into Essential thrombocytosis eMedicine med/2266 acute myeloid leukemia or MeSH D013920 Epidemiology myelofibrosis. It is one of four Pathophysiology myeloproliferative disorders. Clinical features Epidemiology Diagnostic criteria Treatment Essential thrombocytosis is diagnosed at a rate of about 2 to 3 per 100,000 individuals Prognosis annually. [1][2] The disease usually affects middle aged to elderly individuals, with an Special care related to pregnancy average age at diagnosis of 50–60 years, although it can affect children and young References adults as well. [3] External links Images Pathophysiology The pathologic basis for this disease is unknown. However, essential thrombocytosis resembles polycythemia vera in that cells of the megakaryocytic series are more sensitive to growth factors. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis. view all 24 In 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups [4][5][6] to be associated with essential thrombocytosis in around 30% of cases. JAK2 is a member of the Janus kinase family. This mutation may be helpful in making a diagnosis or as a target for future therapy. Clinical features The major symptoms are bleeding and thrombosis. Other symptoms include epistaxis (nosebleeds) and bleeding from gums and gastrointestinal tract. One characteristic symptom is throbbing and burning of the hands and feet due to the occlusion of small arterioles by platelets (erythromelalgia). An enlarged spleen (splenomegaly) may be found on examination. Diagnostic criteria The diagnosis of essential thrombocytosis requires the presence of a persistent thrombocytosis of greater than 600 x109 /L in the absence of an alternative cause. The following revised diagnostic criteria for essential thrombocytosis were proposed in 2005 [7] . The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6. A1. Platelet count > 600 x 10 9 /L for at least 2 months A2. Acquired V617F JAK2 mutation present B1. No cause for a reactive thrombocytosis normal inflammatory indices B2. No evidence of iron deficiency stainable iron in the bone marrow or normal red cell mean corpuscular volume B3. No evidence of polycythemia vera
  6. 6. hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores B4. No evidence of chronic myeloid leukemia But the Philadelphia chromosome may be present in up to 10% of cases. Patients with the Philadelphia chromosome have a potential for the development of acute leukemia, especially acute lymphocytic leukemia. B5. No evidence of myelofibrosis no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0–4 scale) B6. No evidence of a myelodysplastic syndrome no significant dysplasia no cytogenetic abnormalities suggestive of myelodysplasia Treatment Not all patients will require treatment at presentation. In those who are at increased risk of thrombosis or bleeding (older age, prior history of bleeding or thrombosis, or very high platelet count), reduction of the platelet count to the normal range can be achieved using hydroxyurea (also known as hydroxycarbamide), interferon-α or anagrelide. Low- dose aspirin is widely used to reduce the risk of thrombosis, but there may be an increased risk of bleeding if aspirin is initiated while the platelet count is very high. The PT1 study [8] compared hydroxyurea in combination with aspirin to anagrelide in combination with Aspirin as initial therapy for essential thrombocytosis. Hydroxyurea was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and lower risk of transformation to myelofibrosis (although the rate of venous thrombosis was higher with hydroxycarbamide than with anagrelide). In rare cases where patients have life-threatening complications, the platelet count can be reduced rapidly using platelet apheresis (a procedure that removes platelets from the blood directly). ... Prognosis Essential thrombocytosis is sometimes described as a slowly progressive disorder with long asymptomatic periods punctuated by thrombotic or hemorrhagic events. However, well-documented medical regimes can reduce and control the number of platelets, which reduces the risk of these thrombotic or haemorrhagic events. The lifespan of a well controlled ET person is well within the expected range for a person of similar age but without ET. Special care related to pregnancy Hydroxyurea and anagrelide are contraindicated during pregnancy and nursing. There is current debate as to the safety of interferon during pregnancy and nursing. Essential thrombocytosis can be linked with increased risk of spontaeous abortion or miscarriage in the first trimester of pregnancy. Throughout pregnancy, close monitoring of the mother for thrombosis and placenta is recommended to ensure blood clots are caught. Post partum, often daily injections of low dose low molecular weight heparin (e.g. enoxaparin) are prescribed for several weeks as this is a period where the mother is at higher risk of developing a blood clot. References 1. ↑ Mesa R, Silverstein M, Jacobsen S, Wollan P, Tefferi A (1999). "[Expression error: Missing operand for > Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995]". Am J Hematol 61 (1): 10–5. doi:10.1002/(SICI)1096- 8652(199905)61:1<10::AID-AJH3>3.0.CO;2-I. PMID 10331505. 2. ↑ Kutti J, Ridell B (2001). "[Expression error: Missing operand for > Epidemiology of the myeloproliferative disorders: essential thrombocythaemia, polycythaemia vera and idiopathic myelofibrosis]". Pathol Biol (Paris) 49 (2): 164–6. PMID 11317963. 3. ↑ Hoffman: Hematology: Basic Principles and Practice, 4th ed., 2005 Churchill Livingstone, Chapter 71. 4. ↑ Kralovics R, Passamonti F, Buser AS, Teo SS, et al. (2005). "[Expression error: Missing operand for > A gain-of-function mutation of JAK2 in myeloproliferative disorders]". N Engl J Med 352 (17): 1779–90. doi:10.1056/NEJMoa051113. PMID 15858187. 5. ↑ Baxter EJ, Scott LM, Campbell PJ, et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet 365 (9464): 1054–61. doi:10.1016/S0140-6736(05)71142-9. PMID 15781101. http://linkinghub.elsevier.com/retrieve/pii/S0140-6736(05)71142-9.
  7. 7. 6. ↑ Levine RL, Wadleigh M, Cools J, et al. (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell 7 (4): 387–97. doi:10.1016/j.ccr.2005.03.023. PMID 15837627. http://linkinghub.elsevier.com/retrieve/pii/S1535-6108(05)00094-2. 7. ↑ Campbell PJ, Green AR (2005). "Management of polycythemia vera and essential thrombocythemia". Hematology Am Soc Hematol Educ Program 2005: 201–8. doi:10.1182/asheducation-2005.1.201. PMID 16304381. http://www.asheducationbook.org/cgi/pmidlookup?view=long&pmid=16304381. 8. ↑ Harrison CN, Campbell PJ, Buck G, et al. (2005). "Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia". N. Engl. J. Med. 353 (1): 33–45. doi:10.1056/NEJMoa043800. PMID 16000354. http://content.nejm.org/cgi/pmidlookup?view=short&pmid=16000354&promo=ONFLNS19. External links Cancerbackup Essential Thrombocytosis page CMPD Education Foundation Pathology: hematology · hematologic diseases of RBCs and megakaryocytes / show MEP (D50-69,74, 280-287) Categories: Hematology | Blood disorders History View article history All Wikipedia content is licensed under the GNU Free Document License or the Creative Commons CC-BY-SA license or is otherwise used here in compliance with the Copyright Act Go to Bing in English © 2010 Microsoft | Προστασία προσωπικών δεδομένων | Νομικές ανακοινώσεις | Βοήθεια

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