Chronic Myelomonocytic Leukemia: Natural History and Prognostic Determinants
Section snippets
METHODS
Between 1980 and 1988, 41 patients who fulfilled the FAB criteria for CMML (all patients had dysplastic marrow containing less than 20% blast cells and a peripheral monocyte count of more than 1.0 × 109/liter; excluded were patients who had more than 5% blast cells in the peripheral blood and Auer rods in the leukemic blast cells) were examined at the Mayo Clinic. The histories of these patients along with additional prereferral and follow-up information were reviewed retrospectively.
In each
RESULTS
The median age of our 41 study patients at the time of diagnosis of CMML was 66 years (range, 1 to 84 years). The two youngest patients were 1 and 36 years old. In contrast to previous reports,10, 12 our study group had a definite male predilection—2.4:1. Most patients either were asymptomatic or had symptoms of anemia at the time of initial examination. Unusual manifestations included postbath pruritus (in two patients) and relapsing polychondritis, acquired paroxysmal nocturnal
DISCUSSION
CMML mimics the chronic myeloproliferative diseases in displaying increased peripheral and marrow hyperleukocytosis, a high frequency of hepatosplenomegaly, and occasional marrow fibrosis. In fact, two of our patients had a previous diagnosis of agnogenic myeloid metaplasia, and seven bone marrow slides were primarily interpreted as chronic granulocytic leukemia before the results of the chromosome studies became available. The latter are sometimes misdiagnosed as Ph1-negative chronic
CONCLUSION
The percentage of blast cells in the bone marrow seems to be the single most important prognostic determinant in CMML. Alternatively, the “modified Bournemouth score” and the hemoglobin level offer significant but not independent prognostic information. Adequacy in the number of patients being reported, strict adherence to the FAB criteria during selection of patients, and stratification of patients on the basis of bone marrow blast cell percentage may reduce the degree of discrepancy in
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