Case Report

Juvenile Myelomonocytic Leukemia in a Child: A Case Report of Palliative Chemotherapy and Literature Review Applied to Limited Resources Centers

Table 2

Diagnosis criteria for juvenile myelomonocytic leukemia (JMML) per the 2016 revision to the World Health Organization classification [10].

Category 1 (all are required)Clinical and hematologic featuresAbsence of the BCR-ABL1 fusion gene
>1 × 10/L circulating monocytes
<20% blasts in the peripheral blood and bone marrow
Splenomegaly

Category 2 (one is sufficient)Genetic studiesSomatic mutation in KRAS, NRAS, or PTPN11 (germline mutations need to be excluded)
Clinical diagnosis of NF-1 or NF-1 gene mutation
Germline CBL mutation and loss of heterozygosity of CBL

Category 3 (patients without genetic features must have the following in addition to category 1)Other featuresMonosomy 7 or other chromosomal abnormality or at least 2 of the criteria given as follows:
circulating myeloid or erythroid precursors,
increased hemoglobin F for age,
hyperphosphorylation of STAT-5,
GM-CSF hypersensitivity

NF-1, neurofibromin-1; CBL, casitas B-lineage lymphoma; PTPN11, protein tyrosine phosphatase nonreceptor type; KRAS, Kirsten rat sarcoma; NRAS, neuroblastoma rat sarcoma.