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.