chronic myelomonocytic leukemia (CMML)
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Introduction
Two subtypes:
- patients with WBC< or = 12,000/uL, considered myelodysplastic syndrome
- patients with WBC > 12,000/uL, considered chronic myeloproliferative disorder
Epidemiology
- 16% of cases of myelodysplastic syndrome
Pathology
- circulating blasts < 5%
- bone marrow blasts 1-20%
- < 15% ringed sideroblasts in marrow
- no Auer rods
- monocytes > 1 x 10E09/L (1000/uL)
Genetics
- t(5;7)(q33;q11.2) involving HIP1 with PDGFRB
- inv(21)(q21;q22) involving USP16 with RUNX1/AML1
Management
- prognosis:
- 3-55% (mean 22%) transformation to acute leukemia (AML)
- survival: 8-60+ months (mean 22 months)
- adverse prognostic factors
- absolute lymphocyte count > 2,500/uL
- hemoglobin below 12 g/dL
- circulating immature myeloid cells
- marrow blasts > 10%
- low percentage of marrow erythroid cells
- high serum lactate dehydrogenase and beta2-microglobulin
- p53 mutation or loss of heterozygosity
More general terms
More specific terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 677-78
- ↑ Onida F et al. Prognostic factors and scoring systems in chronic myelomonocytic leukemia: a retrospective analysis of 213 patients. Blood 99:840-9, 2002 PMID: https://www.ncbi.nlm.nih.gov/pubmed/11806985