Elsevier

Mayo Clinic Proceedings

Volume 64, Issue 10, October 1989, Pages 1246-1254
Mayo Clinic Proceedings

Chronic Myelomonocytic Leukemia: Natural History and Prognostic Determinants

https://doi.org/10.1016/S0025-6196(12)61287-7Get rights and content

A retrospective clinical review of 41 patients with chronic myelomonocytic leukemia revealed a median age of 66 years and a male:female ratio of 2.4:1. The disease was preceded by a myelodysplastic syndrome of a different subtype in 24% of the patients and transformed into acute leukemia in 24%. Splenomegaly was present in 54% of the patients and reached massive proportions in 24%. Chromosomal abnormalities occurred in 34% of those studied, most commonly in the younger age group; the most frequent were trisomy 8, monosomy 7, and deletions involving the long arms of chromosomes 20 and X. Polyclonal hypergammaglobulinemia was detected in 47% of the patients in whom serum protein electrophoresis was done. The median survival was 3 years. With use of univariate analysis, the statistically significant prognostic determinants were hemoglobin level, the “modified Bournemouth score,” and bone marrow blast cell percentage. When these factors were subjected to a multivariate analysis, only bone marrow blast cell percentage was an independent prognostic determinant. Orally administered hydroxyurea controlled leukocytosis and splenomegaly in some patients without affecting the overall prognosis.

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

REFERENCES (36)

  • P Solal-Celigny et al.

    Chronic myelomonocytic leukemia according to FAB classification: analysis of 35 cases

    Blood

    (1984)
  • D Holden et al.

    Paroxysmal nocturnal hemoglobinuria with acute leukemia

    Blood

    (1969)
  • RH Knapp et al.

    Cytogenetic studies in 174 consecutive patients with preleukemic or myelodysplastic syndromes

    Mayo Clin Proc

    (1985)
  • R Zittoun et al.

    Les leucémies myélo-monocytaires subaiguës: étude de 27 cas et revue de la littérature

    Sem Hop Paris

    (1972)
  • JM Bennett et al.

    Proposals for the classification of the myelodysplastic syndromes

    Br J Haematol

    (1982)
  • B Coiffier et al.

    Dysmyelopoietic syndromes: a search for prognostic factors in 193 patients

    Cancer

    (1983)
  • H Kerkhofs et al.

    Utility of the FAB classification for myelodysplastic syndromes: investigation of prognostic factors in 237 cases

    Br J Haematol

    (1987)
  • M Van der Weide et al.

    Myelodysplastic syndromes: analysis of morphological features related to the FAB-classification

    Eur J Haematol

    (1988)
  • J-M Ribera et al.

    A multivariate analysis of prognostic factors in chronic myelomonocytic leukaemia according to the FAB criteria

    Br J Haematol

    (1987)
  • EP Alessandrino et al.

    Chronic myelomonocytic leukemia: clinical features, cytogenetics, and prognosis in 30 consecutive cases

    Hematol Oncol

    (1985)
  • P Fenaux et al.

    Prognostic factors in adult chronic myelomonocytic leukemia: an analysis of 107 cases

    J Clin Oncol

    (1988)
  • A Worsley et al.

    Prognostic features of chronic myelomonocytic leukaemia: a modified Bournemouth score gives the best prediction of survival

    Br J Haematol

    (1988)
  • AN Stark et al.

    Prognostic factors and survival in chronic myelomonocytic leukaemia (CMML)

    Br J Cancer

    (1987)
  • Group Français de Cytogénétique Hématologique

    Cytogenetics of chronic myelomonocytic leukemia

    Cancer Genet Cytogenet

    (1986)
  • RV Pierre

    Preleukemia/myelodysplasia

    Curr Haematol Oncol

    (1988)
  • G Dewald et al.

    A cytogenetic method for mailed-in bone marrow specimens for the study of hematologic disorders

    Lab Med

    (1982)
  • ISCN

    An International System for Human Cytogenetic Nomenclature

    Birth Defects

    (1985)
  • EL Kaplan et al.

    Nonparametric estimation from incomplete observations

    J Am Stat Assoc

    (1958)
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