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[Clinical course, morphology and prognosis of chronic myelomonocytic leukemia]
U Germing1, C Strupp, G Meckenstock
1Klinik für Hämatologie, Onkologie und klinische Immunologie, Heinrich-Heine-Universität Düsseldorf. germing@haemouk.uni-duesseldorf.de
Insights
The FAB classification distinguishes myelodysplastic (MDS-CMML) and myeloproliferative (MPD-CMML) subtypes of chronic myelomonocytic leukemia. While clinically distinct, these subtypes offer limited prognostic value, though the Düsseldorf score aids risk stratification in CMML.
Area of Science:
- Hematology
- Oncology
- Clinical Medicine
Background:
- The FAB group proposed classifying chronic myelomonocytic leukemia (CMML) into myelodysplastic (MDS-CMML) and myeloproliferative (MPD-CMML) subtypes based on leukocyte count.
- Prognostic factors for CMML subtypes remain incompletely established.
Purpose of the Study:
- To compare clinical and hematological features of MDS-CMML and MPD-CMML.
- To examine the natural disease course and identify prognostic factors in CMML subtypes.
Main Methods:
- Retrospective analysis of 225 CMML patients diagnosed at an institution.
- Comparison of presenting features, disease course, and survival between MDS-CMML and MPD-CMML.
- Evaluation of prognostic factors including the Düsseldorf score.
Main Results:
- MPD-CMML showed higher rates of splenomegaly, hepatomegaly, and LDH values; bone marrow blast percentages and dysplasia signs were similar between subtypes.
- Five-year actuarial survival was 24% for MPD-CMML versus 15% for MDS-CMML.
- MDS-CMML had a higher probability of transformation to acute myeloid leukemia (AML) (29% vs 18% at 5 years).
- Elevated LDH, low hemoglobin, and male sex were independent risk factors for overall and MDS-CMML survival.
- The Düsseldorf score effectively stratified risk within MDS-CMML (median survival 12 vs 40 months).
Conclusions:
- MDS-CMML and MPD-CMML represent clinically distinct conditions, but this classification offers limited prognostic information.
- The Düsseldorf score is a valuable tool for risk stratification in CMML.
- Further research into prognostic factors for MPD-CMML is warranted.
Background:
The FAB group proposed to distinguish 2 subgroups of chronic myelomonocytic leukemia (CMML): Depending on the total leukocyte count a myelodysplastic type (< 13,000/microliter) was separated from a myeloproliferative type (> 13,000/microliter). Prognostic factors are not well-established until now.
Patients And Methods:
Based on retrospective analyses of patients with CMML diagnosed at our institution, we compared the presenting clinical and hematological features of both disorders and examined the natural course of the disease and prognostic factors.
Results:
Out of 225 patients with CMML there were 115 patients with myelodysplastic type (MDS-CMML) and 110 patients with myeloproliferative type (MPD-CMML). Median age of patients at diagnosis and sex ratio were not different. Splenomegaly and hepatomegaly were more common in MPD-CMML. With regard to laboratory findings, patients with MPD-CMML presented with significantly higher LDH values. Except for WBC, peripheral blood counts were not different. Median percentage of bone marrow blasts was 8% in both disorders. Signs of bone marrow dysplasia were comparable in both disorders. Cumulative survival rates were similar in both disorders. Five years after diagnosis, actuarial survival for patients with MPD-CMML was 24%, as compared to 15% for patients with MDS-CMML. The probability of transformation to AML was higher in MDS-CMML (29% vs 18% after 5 years). Elevated LDH values, low hemoglobin values and male sex were independent risk factors for the entire group and for the MDS-CMML group. Using the Düsseldorf score, we could define risk groups within MDS-CMML with a median survial of 12 vs 40 months (p = 0.00005). Prognostic factors could not define risk groups within the MPD-CMML group.
Conclusions:
In summary, these data suggest that MDS-CMML and MPD-CMML are clinically distinguishing conditions, but the separation provides little prognostic information. The Düsseldorf score can be used to provide risk stratification in CMML.