WHO diagnostic criteria pre-primary myelofibrosis

Major criteria

Megakaryocytic proliferation and atypia, without reticulin fibrosis >grade 1, accompanied by increased age-adjusted bone marrow cellularity, granulocytic proliferation, and often decreased erythropoiesis

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

Presence of JAK2, CALR, or MPL mutation or in the absence of these mutations, presence of another clonal marker,* or absence of minor reactive bone marrow reticulin fibrosis^¶

Minor criteria

Presence of at least 1 of the following, confirmed in 2 consecutive determinations:

Anemia not attributed to a comorbid condition

Leukocytosis ≥11 × 10⁹/L

Palpable splenomegaly

LDH increased to above upper normal limit of institutional reference range

Diagnosis of prePMF requires meeting all 3 major criteria, and at least 1 minor criterion

CML: chronic myeloid leukemia; ET: essential thrombocythemia; PMF: primary myelofibrosis; PV: polycythemia vera; WHO: World Health Organization
* In the absence of any of the 3 major clonal mutations, the search for the most frequent accompanying mutations (eg, ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1) are of help in determining the clonal nature of the disease.
¶ Minor (grade 1) reticulin fibrosis secondary to infection, autoimmune disorder or other chronic inflammatory conditions, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies.

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 pre-primary myelofibrosis