WHO diagnostic criteria overt primary myelofibrosis

Major criteria

Presence of megakaryocytic proliferation and atypia, accompanied by either reticulin and/or collagen fibrosis grades 2 or 3

Not meeting WHO criteria for ET, PV, BCR-ABL1⁺ CML, 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 reactive myelofibrosis^¶

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

Leukoerythroblastosis

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

CML: chronic myeloid leukemia; ET: essential thrombocythemia; LDH: lactate dehydrogenase; PMF: primary myelofibrosis; PV: polycythemia vera
* 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.
¶ Bone marrow 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 overt primary myelofibrosis