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chronic myelomonocytic leukemia (CMML)
Two subtypes:
1) patients with WBC< or = 12,000/uL, considered myelodysplastic syndrome
2) patients with WBC > 12,000/uL, considered chronic myeloproliferative disorder
Epidemiology:
-> 16% of cases of myelodysplastic syndrome
Pathology:
1) circulating blasts < 5%
2) bone marrow blasts 1-20%
3) < 15% ringed sideroblasts in marrow
4) no Auer rods
5) 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:
a) 3-55% (mean 22%) transformation to acute leukemia (AML)
b) survival: 8-60+ months (mean 22 months)
c) 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
Interactions
disease interactions
Specific
chronic myelomonocytic leukemia/t[5/12]
General
chronic leukemia
chronic myeloproliferative disorder
myelodysplastic syndrome (MDS)
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: 11806985