Polycythemia vera classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2] Mohamad Alkateb, MBBCh [3]; Shyam Patel [4]


Polycythemia vera is a subtype of myeloproliferative neoplasm. Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes: chronic myelogenous leukemia, chronic neutrophilic leukemia, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic eosinophilic leukemia, mastocytosis, and myeloproliferative neoplasms, unclassifiable. The classification of polycythemia is subdivided into primary polycythemia (which is a clonal process caused by the JAK2 mutation) and secondary polycythemia (which is a reactive process due to a state of chronic hypoxia). There are numerous causes of secondary polycythemia, and most of these causes are cardiopulmonary in origin.


Classification of myeloproliferative neoplasms

Polycythemia vera is a subtype of myeloproliferative neoplasm. Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes:[1][2][3][4][5][6][7][8][9][10]

There are no subcategories within polycythemia vera.

Classification of polycythemia

Polycythemia vera is a subcategory of polycythemia in general. Classification of polycythemia in general includes primary polycythemia and secondary polycythemia. Secondary polycythemia is due to chronic hypoxia which results in compensatory increase in erythrocyte production. Secondary polycythemia is therefore characterized by a reactive increase in erythrocyte production, rather than clonal proliferation of erythrocytes.


  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. Birgegård G (2015). "Advances and challenges in the management of essential thrombocythemia". Ther Adv Hematol. 6 (3): 142–56. doi:10.1177/2040620715580068. PMC 4480522. PMID 26137205.
  4. Beatrice JM, Garanito MP (2013). "Essential thrombocythemia: a rare disease in childhood". Rev Bras Hematol Hemoter. 35 (4): 287–9. doi:10.5581/1516-8484.20130059. PMC 3789436. PMID 24106449.
  5. Hayashi Y, Hirai H, Kamio N, Yao H, Yoshioka S, Miura Y; et al. (2013). "C/EBPβ promotes BCR-ABL-mediated myeloid expansion and leukemic stem cell exhaustion". Leukemia. 27 (3): 619–28. doi:10.1038/leu.2012.258. PMC 4506742. PMID 22948537.
  6. Gotlib J (2017). "How I treat atypical chronic myeloid leukemia". Blood. 129 (7): 838–845. doi:10.1182/blood-2016-08-693630. PMID 27899359.
  7. Maxson JE, Tyner JW (February 2017). "Genomics of chronic neutrophilic leukemia". Blood. 129 (6): 715–722. doi:10.1182/blood-2016-10-695981. PMC 5301820. PMID 28028025.
  8. Desterke C, Martinaud C, Ruzehaji N, Le Bousse-Kerdilès MC (2015). "Inflammation as a Keystone of Bone Marrow Stroma Alterations in Primary Myelofibrosis". Mediators Inflamm. 2015: 415024. doi:10.1155/2015/415024. PMC 4660030. PMID 26640324.
  9. Vidyadharan S, Joseph B, Nair SP (2016). "Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations". Indian J Dermatol. 61 (4): 437–9. doi:10.4103/0019-5154.185716. PMC 4966405. PMID 27512192.
  10. Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O; et al. (2017). "Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future". Cancer Res. 77 (6): 1261–1270. doi:10.1158/0008-5472.CAN-16-2234. PMC 5354959. PMID 28254862.