Table 1.
WHO diagnostic criteria for CNL and aCML, with corresponding patient clinical/laboratory data.
| WHO DIAGNOSTIC CRITERIA | PATIENT DATA | COMPARISON | ||
|---|---|---|---|---|
| aCML | CNL | CNL (√/X) | ACML (√/×) | |
| WBCs ≥13 × 109/L with dysgranulopoiesis | WBCs ≥25 × 109/L with segmented neutrophils >80% of WBCsa | WBCs 40.9 × 109/L ith >80% neutrophils and no dysgranulopoiesis | √ | × |
| Hypercellularmarrowb | Hypercellularmarrowc | Hypercellular marrow with mature forms | √ | √ |
| No Ph or BCR-ABL1 fusion gene | No Ph or BCR-ABL1 fusion gene | No Ph or BCR-ABL1 fusion gene | √ | √ |
| No rearrangement PDGFRα/β | No rearrangement PDGFRα/β or FGFR1 | No rearrangement PDGFRα/β or FGFR1 | √ | √ |
| Blood neutrophil precursors ≥10% of WBCs | Hepatosplenomegaly | Blood neutrophil precursors <10% WBCs | √ | × |
| Minimal basophilia (<2%) | No physiologic cause for neutrophilia | No basophilia in blood or marrow | √ | √ |
| Minimal monocytosis (<10%) | No evidence of PV, ET, or PM | Monocytes <1% | √ | √ |
| Less than 20% blasts in blood and marrow | No evidence of MDS or MDS/MPD | Less than 20% blasts in blood and marrow | √ | √ |
| Hepatosplenomegaly (mild) | √ | × | ||
| No physiologic cause for neutrophilia | √ | √ | ||
| No evidence of PV, ET, or PM | √ | √ | ||
| No evidence of MDS or MDS/MPD | √ | √ | ||
Note:
Segmented neutrophils and band forms are >80% of WBCs, immature granulocytes <10% of WBCs, and myeloblasts <1% of WBCs.
Granulocytic proliferation and granulocytic dysplasia with or without dysplasia in the erythroid and megakaryocytic lineages.
Neutrophilic granulocytes increased in percentage and number, with myeloblasts <5% of nucleated marrow cells, normal neutrophil maturation pattern, and megakaryocytes normal or left shifted.1
Abbreviations: WHO, World Health Organization; CNL, chronic neutrophilic leukemia; aCML, atypical chronic myelogenous leukemia, BCR-ABL1 negative; WBC, white blood cell; Ph, Philadelphia chromosome; PDGFR, platelet-derived growth factor receptor; FGFR, fibroblast growth factor receptor; PV, polycythemia vera; ET, essential thrombocythemia; PM, primary myelofibrosis; MDS, myelodysplastic syndrome; MPD, myeloproliferative disorder; v, patient meets criterion; X, patient does not meet criterion.