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Cytogenetic findings and clinical features in acute leukemia and transient
myeloproliferative disorder in Down's syndrome
Y Hayashi, M Eguchi, K Sugita, S Nakazawa, T Sato, S Kojima, F Bessho, S Konishi, T Inaba and R Hanada
Division of Hematology/Oncology, Saitama Children's Medical Center, Japan.
Cytogenetic, immunologic, and electron microscopic studies were performed
on the blast cells of 28 pediatric patients with Down's syndrome, 13 with
acute leukemia (DS-AL) and 15 with transient myeloproliferative disorders
(DS-TMD). Clonal chromosome abnormalities were found in the cells of all
patients with DS-AL but not those with DS-TMD. The younger ages and higher
hemoglobin concentrations, platelet counts, and WBC counts of DS-TMD
patients provided a clinical contrast with the frankly leukemic cases.
Myelodysplastic syndrome, characterized by a small percentage of leukemic
blast cells, was observed in 11 of the 13 patients with DS-AL compared with
none in the DS-TMD group. Electron microscopy disclosed a positive platelet
peroxidase reaction in each of the 11 DS-TMD patients and in nine of the 13
DS-AL patients. Immunologic studies revealed antiplatelet- megakaryocyte
antigens on the blast cells of the majority of patients in both study
groups. Our findings suggest that the blast cells in cases of DS-AL and
DS-TMD arise from cells of the megakaryocytic lineage or from a myeloid
progenitor with the capacity for megakaryocytic differentiation. The high
risk of the development of AL in patients with DS who are less than 3 years
old may be related to increased megakaryocyte proliferation in this age
group.
Volume 72,
Issue 1,
pp. 15-23,
07/01/1988
Copyright © 1988 by The American Society of Hematology
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