Distinctive Demography, Biology, and Outcome of Acute Myeloid Leukemia and Myelodysplastic Syndrome in Children With Down Syndrome: Children's Cancer Group Studies 2861 and 2891

摘要: In recent pediatric trials of acute myeloid leukemia (AML), children with Down syndrome (DS) have had significantly more megakaryoblastic and experienced better outcome than other children. To further characterize AML in DS, Children's Cancer Group Studies 2861 2891 prospectively studied demography, biology, response myelodysplastic (MDS) without DS. These studies evaluated timing induction therapy compared postremission chemotherapy marrow transplantation 1,206 One-hundred eighteen (9.8%) a fourfold increase 20 years. DS patients were younger, lower white blood cell platelet counts, antecedent MDS, or undifferentiated AML, an under-representation chromosomal translocations ( P < .001 for each variable). Four-year event-free survival was 69% versus 35% others .001). Intensively timed conferred higher mortality patients; bone offered no advantage. Conventional followed by achieved 88%, 4-year, disease-free 42% Megakaryoblastic unfavorable but prognostically neutral is demographically biologically distinct from It singularly responsive to conventional may warrant even less therapy. The increasing proportion most likely reflects changes attitudes about entering on possibly ability distinguish lymphoid leukemia.