Acute megakaryoblastic leukemia in early childhood
WC Chan, RK Brynes, TH Kim, A Verras, C Schick, RJ Green and AH Ragab
Two girls, each less than 2 yr of age, developed acute megakaryoblastic
leukemia (malignant myelosclerosis). Both presented with anemia, severe
thrombocytopenia, and a low percentage of blasts in their peripheral blood.
Their marrow showed marked reticulin fibrosis with an increase in blasts
and immature megakaryocytes. The blasts stained negatively for
myeloperoxidase and Sudan Black B, but showed acid phosphatase (ACP) and
alpha-naphthyl acetate esterase (ANAE) activity inhibitable by sodium
fluoride. They were identified as megakaryoblasts by the platelet
peroxidase reaction. Cytogenetic studies showed multiple chromosomal
abnormalities in both cases. Chemotherapy with vincristine, prednisone, and
L-asparaginase was without effect, while daunorubicin and cytosine
arabinoside induced a complete remission in one case. The second case
responded to a combination of cytosine arabinoside, daunorubicin, and
6-thioguanine. This article documents that acute megakaryoblastic leukemia
occurs in early childhood and describes its clinical, pathologic, and
cytogenetic features. Previous reports of childhood "myelofibrosis" are
reviewed, and their possible relationship with acute megakaryoblastic
leukemia is discussed.
Volume 62,
Issue 1,
pp. 92-98,
07/01/1983
Copyright © 1983 by The American Society of Hematology
