WHO Diagnostic Criteria

Patient Data

Comparison

aCML

CNL

CNL

(√/X)

aCML

(√/X)

WBCs ≥ 13x109/L with dysgranulopoiesis

WBCs ≥ 25x109/L with segmented neutrophils >80% of WBCsa

WBCs 125x109/L with >80% neutrophils and no dysgranulopoiesis

X

Hypercellular marrowb

Hypercellular marrowc

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

X

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 3%

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)

X

No physiologic cause for neutrophilia

No evidence of PV, ET, or PM

No evidence of MDS or MDS/MPD

Table 1: World Health Organization (WHO) diagnostic criteria for chronic neutrophilic leukemia (CNL) and atypical chronic myelogenous leukemia (aCML) with corresponding patient clinical/laboratory data.
 
Abbreviations: WHO = World Health Organization; CNL = chronic neutrophilic leukemia; aCML = atypical chronic myelogenous leukemia, BCR-ABL1 negative; WBC = white blood cell; Ph = Philadelphia chromosome; PDGRF = 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.
 
a. segmented neutrophils and band forms are >80% of WBCs, immature granulocytes <10% of WBCs, and myeloblasts <1% of WBCs. b. granulocytic proliferation and granulocytic dysplasia with or without dysplasia in the erythroid and megakaryocytic lineages. c. 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].