Myeloproliferative neoplasm overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The myeloproliferative neoplasm are a group of diseases of the bone marrow in which excess cells are produced. Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes (chronic myelogenous leukemia, BCR-ABL1–positive, chronic neutrophilic leukemia, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic eosinophilic leukemia, not otherwise specified, mastocytosis, myeloproliferative neoplasms, unclassifiable).[1][2] They are related to, and may evolve into, myelodysplastic syndrome and acute myeloid leukemia, although the myeloproliferative diseases on the whole have a much better prognosis than these conditions. Clinical and pathologic features in the myeloproliferative neoplasms are due to dysregulated proliferation and expansion of myeloid progenitors in the bone marrow, resulting in altered populations of granulocytes, erythrocytes, or platelets in the peripheral blood. Myeloproliferative neoplasm is caused by a mutation in the BCR-ABL, Janus kinase 2, and calreticulin genes.[3][4] Patients may be asymptomatic at diagnosis. However, if symptoms present, symptoms of myeloproliferative neoplasm include fever, fatigue, and bleeding.[5][6]

References

  1. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A; et al. (2009). "The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes". Blood. 114 (5): 937–51. doi:10.1182/blood-2009-03-209262. PMID 19357394.
  2. Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB; et al. (2001). "Diagnostic criteria and classification of mastocytosis: a consensus proposal". Leuk Res. 25 (7): 603–25. PMID 11377686.
  3. Ganfyd. Polycythaemia vera 2015.http://www.ganfyd.org/index.php?title=Polycythemia_vera
  4. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID http://dx.doi.org/10.1182/blood-2013-11-538983 Check |pmid= value (help).
  5. Agarwal MB, Malhotra H, Chakrabarti P, Varma N, Mathews V, Bhattacharyya J; et al. (2015). "Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythemia vera, and essential thrombocythemia". Indian J Med Paediatr Oncol. 36 (1): 3–16. doi:10.4103/0971-5851.151770. PMC 4363847. PMID 25810569.
  6. Canadian Cancer Society.2015.http://www.cancer.ca/en/cancer-information/cancer-type/leukemia-chronic-myelogenous-cml/signs-and-symptoms/?region=ab

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