IFN 信号通路发育差异可影响GATA1引发巨核细胞过度增殖

Introduction

Individuals with Down syndrome (DS) have a 10- to 20-fold higher overall risk of leukemia than do non-DS individuals (1–3). A substantial proportion of these excess cases is due to acute megakaryoblastic leukemia (DS-AMKL) during early childhood. DS-AMKL is often anteceded by a transient myeloproliferative disorder (TMD) in the neonatal period that is unique to DS (referred to herein as DS-TMD) and occurs in about 10% of DS newborns (4). The predominant hyperproliferative cells in DS-TMD exhibit erythromegakaryocytic features (5–7). DS-TMD shows a variety of clinical phenotypes, ranging from asymptomatic to respiratory distress and fulminant hepatic failure due to liver f