Advances in Down syndrome related hematopoietic system diseases / 国际儿科学杂志

ABSTRACT

Newborns and children with Down syndrome ( DS) often prensent with transient abnormal myelopoiesis ( TAM) and have an increased risk of acute leukaemia. Most children are able to spontaneously resolve, but early mortality is high, and recurrence of acute megakaryoblastic leukemia (AMKL) occur in about 20% infants within 4 years. Sympotomatic babies with TAM may benefit from low-dose cytarabine. Children with DS are more likely to develop AMKL. DS-AMKL is sensitive to cytarabine and anthracyclines, thus having a superior outcome when compared with non-DS AMKL Age, sex, white blood cell count and risk group are similar between DS-ALL and non-DS-ALL, but DS-ALL has a unique spectrum of sentinel cytogenetic lesions that influences treatment outcome. Risk-adjusted intensive chemotherapy protocols demonstrate a good survival.