WHO diagnostic criteria atypical chronic myeloid leukemia, BCR-ABL1 negative

Peripheral blood leukocytosis due to increased numbers of neutrophils and their precursors (promyelocytes, myelocytes, metamyelocytes) comprising ≥10% of leukocytes

Dysgranulopoiesis, which may include abnormal chromatin clumping

No or minimal absolute basophilia; basophils usually <2% of leukocytes

No or minimal absolute monocytosis; monocytes <10% of leukocytes

Hypercellular bone marrow with granulocytic proliferation and granulocytic dysplasia, with or without dysplasia in the erythroid and megakaryocytic lineages

<20% blasts in the blood and bone marrow

No evidence of PDGFRA, PDGFRB, or FGFR1 rearrangement, or PCM1-JAK2

Not meeting WHO criteria for BCR-ABL1⁺ CML, PMF, PV, or ET*

Diagnosis of atypical CML, BCR-ABL1- requires meeting all of the criteria above.

aCML: atypical chronic myeloid leukemia; CML: chronic myeloid leukemia; CNL: chronic neutrophilic leukemia; ET: essential thrombocythemia; MPN: myeloproliferative neoplasm; PMF: primary myelofibrosis; PV: polycythemia vera; WHO: World Health Organization
* Cases of MPN, particularly those in accelerated phase and/or in post-polycythemic or post-essential thrombocythemic myelofibrosis, if neutrophilic, may simulate aCML. A previous history of MPN, the presence of MPN features in the bone marrow and/or MPN-associated mutations (in JAK2, CALR, or MPL) tend to exclude a diagnosis of aCML. Conversely, a diagnosis of aCML is supported by the presence of SETBP1 and/or ETNK1 mutations. The presence of a CSF3R mutation is uncommon in aCML and if detected should prompt a careful morphologic review to exclude an alternative diagnosis of CNL or other myeloid neoplasm.

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 atypical chronic myeloid leukemia, BCR-ABL1 negative