Ten children (five boys and five girls) with juvenile chronic myelocyt
ic leukemia were seen over a period of 12 years (1980-1991) at the All
India Institute of Medical Sciences, New Delhi. With the exception of
one who was aged 4.5 years, all children were below 4 years of age (m
ean age 20.4 months). The presenting features included fever, bleeding
secondary to thrombocytopenia, marked hepatosplenomegaly, and skin ra
sh. The striking hematological features were anemia, thrombocytopenia,
peripheral blood monocytosis, and normoblastemia. There was no signif
icant myeloid proliferation in the bone marrow aspirate (mean M:E = 5:
1), while erythroid proliferation was prominent along with monocytosis
(mean 11.2%). Fetal hemoglobin was raised in 8 of the 10 patients (me
an 14.1%). Longterm survival was poor, with maximum survival being 18
months in one case. New modalities of management of this rare entity a
re discussed. (C) 1995 Wiley-Liss, Inc.