WHO diagnostic criteria essential thrombocythemia

Major criteria

Platelet count ≥450 × 10⁹/L

Bone marrow biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei. No significant increase or left shift in neutrophil granulopoiesis or erythropoiesis and very rarely minor (grade 1) increase in reticulin fibers.

Not meeting WHO criteria for BCR-ABL1⁺ CML, PV, PMF, myelodysplastic syndromes, or other myeloid neoplasms

Presence of JAK2, CALR, or MPL mutation

Minor criterion

Presence of a clonal marker or absence of evidence for reactive thrombocytosis

Diagnosis of ET requires meeting all 4 major criteria or the first 3 major criteria and the minor criterion

CML: chronic myeloid leukemia; ET: essential thrombocythemia; PMF: primary myelofibrosis; PV: polycythemia vera; WHO: World Health Organization

Republished with permission of the American Society of Hematology, from Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127:2391. Copyright © 2016; permission conveyed through Copyright Clearance Center, Inc.

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WHO diagnostic criteria essential thrombocythemia