Diagnostic criteria for juvenile myelomonocytic leukemia (JMML)

European Working Group of MDS in Childhood (EWOG-MDS), 1998

CategoryRequirements
Clinical featuresThe following clinical features are suggestive of JMML (figures in brackets represent percentage of cases with each clinical feature):
  • Hepatosplenomegaly (97%)
  • Lymphadenopathy (76%)
  • Pallor (64%)
  • Fever (54%)
  • Skin rash (36%)
Laboratory featuresALL THREE of the following criteria must be met:
  1. Peripheral blood monocytosis > 1 x109/L
  2. No Philadelphia chromosome or BCR-ABL fusion gene
  3. < 20% myeloblasts or monoblasts in the bone marrow
Further criteriaAT LEAST TWO of the following criteria must be met:
  1. Increased HbF (corrected for age)
  2. Immature myeloid precursors on peripheral blood smear
  3. Peripheral blood white cell count > 10 x109/L
  4. Clonal cytogenetic abnormalities (including monosomy 7)
  5. In vitro hypersensitivity of myeloid progenitors to GM-CSF