Atypical Chronic Myeloid Leukemia aCML

This disease predominantly involves the neutrophilic series as in CML but lacks Ph1 chromosome or bcr/abl translocation.182 aCML has dysplastic and proliferative features as well as an increased monocyte count. Its prognosis is significantly worse than Ph1+ CML. According to recently developed FAB criteria CML, aCML, and CMML can be distinguished from each other by employing five quantitative (WBC count, percentage of immature granulocytes, percentage of monocytes and basophils in peripheral blood, and percentage of erythroid precursors in bone marrow) and one qualitative (granulocytic dysplasia) parameters.183

Juvenile Myelomonocytic Leukemia

JMML is a separate disorder seen in children and adolescents and distinct from adult CMML. Young patient age (median 1.8 years), predominant hepatosplenomegaly, frequent skin involvement, leukocytosis, monocytosis, and presence of immature precursors in peripheral blood characterize JMML. Fourteen percent of patients are known to have neurofibromatosis type I by clinical criteria. The JMML cells demonstrate hypersensitivity to GM-CSF in in vitro dose-response assays. Although 65 percent of patients with JMML have a normal karyotype, 25 percent show monosomy 7 and 10 percent complex chromosomal abnormalities.184,185

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