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Chronic myelomonocytic leukemia in childhood: a retrospective analysis of
110 cases. European Working Group on Myelodysplastic Syndromes in Childhood
(EWOG-MDS)
CM Niemeyer, M Arico, G Basso, A Biondi, A Cantu Rajnoldi, U Creutzig, O Haas, J Harbott, H Hasle, G Kerndrup, F Locatelli, G Mann, B Stollmann-Gibbels, ET van't Veer-Korthof, E van Wering and M Zimmermann
Department of Pediatrics, University of Freiburg, Germany.
Chronic myelomonocytic leukemia (CMML) is a rare hematopoietic malignancy
of childhood. To define the clinical and hematologic characteristics of the
disease, we performed a retrospective analysis of 110 children given the
diagnosis CMML irrespective of karyotype. Median age at diagnosis was 1.8
years. Neurofibromatosis type 1 was known in 14% and other clinical
abnormalities in 7% of the children. At presentation, the medium white
blood count was 35 x 10(9)/L, with a median monocyte count of 7 x 10(9)/L.
Karyotypic abnormalities in bone marrow cells were noted in 36% of the
patients, whereas 26% of the children had monosomy 7. Children with
monosomy 7 did not differ from those with normal karyotype with respect to
their clinical presentation. However, they did display some characteristic
hematologic features. Of 110 children, 38 received an allogeneic bone
marrow transplant (BMT). The probability of survival at 10 years was 0.39
(standard error [SE] = 0.10) for the BMT group and 0.06 (SE = 0.4) for the
72 patients of the non-BMT group. Platelet count, age, and hemoglobin F at
diagnosis were the main predicting factors for the length of survival in
the non-BMT group. There is a strong need for a broad agreement on
nomenclature in children with myelodysplastic syndromes (MDS). We propose
here to use the French-American-British classification for MDS in
childhood.
Volume 89,
Issue 10,
pp. 3534-3543,
05/15/1997
Copyright © 1997 by The American Society of Hematology
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