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: chronic myelogenous leukemia, chronic neutrophilic leukemia, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic eosinophilic leukemia, mastocytosis, and myeloproliferative neoplasms, unclassifiable.

Classification

Disease	Cell of origin	W.H.O. 2016 Diagnostic criteria
Polycythemia vera	Erythroid precursor	Major criteria: Hemoglobin > 16.5 g/dl in men or hemoglobin > 16 g/dl in women Bone marrow biopsy showing hypercellularly for age and trilineage growth (panmyelosis) Presence of JAK2 V617F or exon 12 mutation Minor criterion: Subnormal erythropoietin level Diagnosis requires meeting all 3 major criterion or the top 2 major plus the 1 minor criterion
Essential thrombocythemia	Megakaryocyte	Major criteria: Platelet count > 450,000 per microliter Bone marrow biopsy showing mainly proliferation of megakaryocytes with increased number of enlarged and mature megakaryocytes Not meeting criteria for other myeloproliferative neoplasms Presence of JAK2, CALR, or MPL mutation Minor criterion: Presence of a clonal marker or absence of reactive thrombocytosis 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: Anemia White blood cell count >11,000 per microliter. Palpable splenomegaly Elevated LDH Leukoerythroblastic smear Diagnosis requires meeting all major criteria and at least 1 minor criterion.
Chronic myeloid leukemia	Common myeloid precursor
Chronic neutrophilic leukemia	Neutrophil
Chronic eosinophilic leukemia	Eosinophil
Myeloproliferative neoplasm, unclassifiable	Variable
Mastocytosis	Mast cell