Myeloproliferative neoplasm classification

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

Overview

Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes: polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic myelogenous leukemia, chronic neutrophilic leukemia, chronic eosinophilic leukemia, myeloproliferative neoplasms unclassifiable, and mastocytosis. Each subtypes is based on a distinct malignant cell, and each subtype has different criteria for diagnosis.

Classification

Myeloproliferative neoplasm may be classified according to the World Health Organization into eight subtypes as follows:[1][2][3]

Disease Cell of origin W.H.O. Diagnostic criteria

Polycythemia vera

Erythroid precursor

Major criteria:

Minor criterion:

Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion

Essential thrombocythemia

Megakaryocyte

Major criteria:

Minor criterion:

Diagnosis requires meeting all 4 major criteria or the first 3 major plus the 1 minor criterion

Primary myelofibrosis

Megakaryocyte

Major criteria:

  • Presence of megakaryocyte proliferation and atypia with reticulin fibrosis
  • Not meeting criteria for other myeloproliferative neoplasms
  • Presence of JAK2, CALR, or MPL mutation

Minor criteria:

Diagnosis requires meeting all major criteria and at least 1 minor criterion

Chronic myeloid leukemia

Common myeloid progenitor

Chronic neutrophilic leukemia

Neutrophil

Chronic eosinophilic leukemia

Eosinophil

No formal W.H.O. criteria

Myeloproliferative neoplasm, unclassifiable

Variable

Not meeting criteria for other subcategories

Mastocytosis

Mast cell

Major criteria:

Minor criteria:

Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria

References

  1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM; et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.
  2. 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.
  3. 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.

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