再生障碍性贫血相关信号通路的研究进展

Research advances on signaling pathways related to aplastic anemia

再生障碍性贫血(aplastic anemia,AA)是一类由多种原因引起的全血细胞减少和骨髓增生减低的骨髓衰竭性疾病,临床主要表现为贫血、出血及感染等,按疾病严重程度可分为重型再生障碍性贫血(se-vere aplastic anemia,SAA)和非重型再生障碍性贫血(non-severe aplastic anemia,NSAA)。其中,对于年龄≤35岁的SAA患者,首选人类白细胞抗原(human lymphocyte antigen,HLA)相合的同胞供者异基因造血干细胞移植;35~50岁的SAA患者可优选HLA相合的同胞供者异基因造血干细胞移植,次选免疫抑制治疗(immunosuppressive therapy,IST);而对于年龄>50岁的SAA患者,或NSAA患者,推荐IST。

Aplastic anemia(AA) is a bone marrow failure syndrome,and the pathogenesis is believed to be mainly related to immune abnormality,hematopoietic stem/progenitor cell deficiency and bone marrow hematopoietic microenvironment abnormality.In recent years,studies have reported the role of signaling pathways in the pathogenesis of A A.Due to the complexity of the pathogenesis of AA,there are multiple signaling pathways that participate in development of the disease,mainly including mTOR,Notch1,SDF-1α/CXCR4 and Tim-3 signaling pathways(related to immune abnormality),TPO/c-MPL and Fas/FasL signaling pathways(hematopoietic stem cell apoptosis and defect),and PPARγ,MAPK/ERK and VEGF/Notch1 signaling pathways(related to abnormal hematopoietic microenvironment).This article reviews the signaling pathways involved in the pathogenesis of AA,in order to provide new ideas for the pathogenesis and treatment of A A.