WHO diagnostic criteria for chronic neutrophilic leukemia

Peripheral blood WBC ≥25 × 10⁹/L

Segmented neutrophils plus band forms ≥80% of WBCs

Neutrophil precursors (promyelocytes, myelocytes, and metamyelocytes) <10% of WBC

Myeloblasts rarely observed

Monocyte count <1 × 10⁹/L

No dysgranulopoiesis

Hypercellular bone marrow

Neutrophil granulocytes increased in percentage and number

Neutrophil maturation appears normal

Myeloblasts <5% of nucleated cells

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

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

Presence of CSF3R T618I or other activating CSF3R mutation

In the absence of a CSFR3R mutation, persistent neutrophilia (at least 3 months), splenomegaly and no identifiable cause of reactive neutrophilia including absence of a plasma cell neoplasm or, if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies

The diagnosis of chronic neutrophilic leukemia requires all five criteria.

CML: chronic myeloid leukemia; ET: essential thrombocythemia; PV: polycythemia vera; PMF: primary myelofibrosis; WBC: white blood cell count; 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 for chronic neutrophilic leukemia