INTRODUCTION
Children with Down Syndrome (DS) have a 10- to 20- fold increased risk
of developing acute leukemia. The relative risk of developing acute
megakaryoblastic leukemia (AMKL) is estimated to be 500 times higher in
children with DS than in those without DS.1 The
natural history of leukemia in children with DS suggests that trisomy 21
directly contributes to the malignant transformation of hematopoietic
cells, and somatic mutations of the GATA1 gene have been detected in
nearly all ML-DS cases. Other types of acute leukemia occurs in children
with DS, do not have GATA1 mutation and concers either ALL or AML. Only
ML-DS is highly suspectible to chemotherapy2,3.
In Asia, the overall survival for ML-DS in Japan is approximately
80%.4 Retrospective review from 19 patients with
ML-DS in Hong Kong, showed a favorable 5-years event-free and overall
survival of 89.5% and 89.5%, respectively.5 The
survival rates for children in low- and middle-income countries (LMICs)
remain poor6, but there was no report so far regarding
the AML-DS survival rate in LMICs country in Asia.
Here we reported three cases of ML-DS in limited resources pediatric
oncology center in Manado, Indonesia with an innovative combination of
vincristine, daunorubicin, and cytarabine.