Summary of the diagnostic criteria for CNL and aCML from the 2016 revision of the WHO criteria for myeloid neoplasms and acute leukemia
| . | CNL . | aCML . |
|---|---|---|
| Peripheral blood leukocytosis | WBCs ≥25 × 109/L, ≥80% segmented neutrophils and band, <10% neutrophil precursors, rare myeloblasts, monocytes <1 × 109/L | Neutrophils, promyelocytes, myelocytes, and metamyelocytes ≥10% of leukocytes, no or minimal basophilia and monocytosis, <20% blasts |
| Dysgranulopoiesis | No | Yes |
| Bone marrow hypercellularity | Yes; increased neutrophil granulocytes, normal neutrophil maturation, <5% of nucleated cells are myeloblasts | Yes; granulocytic proliferation and dysplasia are present, <20% blasts |
| Do not meet WHO criteria for other disorders | BCR-ABL1 + CML, PV, ET, PMF | BCR-ABL1 + CML, PV, ET, PMF |
| Exclusionary genetic criteria | No rearrangement of PDGFRA, PDGFRB, or FGFR1; no PCM1-JAK2 | No rearrangement of PDGFRA, PDGFRB, or FGFR1; no PCM1-JAK2 |
| Inclusionary genetic criteria | Presence of an activating CSF3R mutation, such as T618I* |
| . | CNL . | aCML . |
|---|---|---|
| Peripheral blood leukocytosis | WBCs ≥25 × 109/L, ≥80% segmented neutrophils and band, <10% neutrophil precursors, rare myeloblasts, monocytes <1 × 109/L | Neutrophils, promyelocytes, myelocytes, and metamyelocytes ≥10% of leukocytes, no or minimal basophilia and monocytosis, <20% blasts |
| Dysgranulopoiesis | No | Yes |
| Bone marrow hypercellularity | Yes; increased neutrophil granulocytes, normal neutrophil maturation, <5% of nucleated cells are myeloblasts | Yes; granulocytic proliferation and dysplasia are present, <20% blasts |
| Do not meet WHO criteria for other disorders | BCR-ABL1 + CML, PV, ET, PMF | BCR-ABL1 + CML, PV, ET, PMF |
| Exclusionary genetic criteria | No rearrangement of PDGFRA, PDGFRB, or FGFR1; no PCM1-JAK2 | No rearrangement of PDGFRA, PDGFRB, or FGFR1; no PCM1-JAK2 |
| Inclusionary genetic criteria | Presence of an activating CSF3R mutation, such as T618I* |
ET, essential thrombocythemia; PMF, primary myelofibrosis; PV, polycythemia vera.
If no CSF3R mutation is present, the diagnostic criteria for CNL can still be met if there is persistent neutrophilia, splenomegaly, and no evidence of the neutrophilia being a reactive process.