Chronic myelomonocytic leukemia: myeloproliferative variant.

Francesco Onida, Miloslav Beran

Research output: Contribution to journalArticle

Abstract

Chronic myelomonocytic leukemia (CMML) has been classified into a new category of myelodysplastic and myeloproliferative diseases by the last World Health Organization classification of myeloid malignancies. However, a large fraction of patients with CMML show unequivocal prevalence of proliferative features, often with conspicuous leukocytosis and organomegaly, making any distinction with atypical chronic myeloid leukemia problematic. Although transformation into acute myeloid leukemia does not seem to be more frequent, prognosis in the proliferative variant of CMML (MP-CMML) is generally worse compared to dysplastic CMML (MD-CMML). Occurrence of mutations in the NRAS and KRAS oncogenes is significantly higher in MP-CMML compared to MD-CMML, whereas cytogenetic abnormalities seem to be less frequent. Constitutively activated platelet-derived growth factor-b receptor tyrosine kinase caused by specific chromosomal aberrations have been documented in a very small proportion of patients with MP-CMML whose malignant cell proliferation have been shown to be inhibited by imatinib mesylate. Treatment of MP-CMML remains challenging, with no strategy proven effective in prolonging survival. While waiting for novel potential targets by further understanding of cell proliferation molecular pathways, new strategies, including stem cell transplant, should be considered in treating patients with MP-CMML.

Original languageEnglish
Pages (from-to)218-226
Number of pages9
JournalCurrent hematology reports
Volume3
Issue number3
Publication statusPublished - May 2004

    Fingerprint

ASJC Scopus subject areas

  • Hematology

Cite this