Table 1 World Health Organization 2016 Revised Diagnostic Criteria for chronic neutrophilic leukemiaa

From: Chronic neutrophilic leukemia: new science and new diagnostic criteria

1. Peripheral blood WBC ≥25 × 109/L

 Segmented neutrophils plus band forms ≥80% of WBC

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

 Myeloblasts rarely observed

 Monocyte count <1 × 109/L

 No dysgranulopoiesis

2. Hypercellular bone marrow

 Neutrophil granulocytes increased in percentage and number

 Normal neutrophil maturation

 Myeloblasts <5% of nucleated cells

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

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

5. Presence of CSF3RT618I or other activating CSF3R mutation

or

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

  1. WBC white blood cells, CML chronic myeloid leukemia, PV polycythemia vera, ET essential thrombocythemia, PMF primary myelofibrosis
  2. a adapted from Arber et al. 38
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