摘要
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.
作者
张小敏
朱玲玲
肖扬
ZHANG Xiao-min;ZHU Ling-ling;XIAO Yang(Jinshazhou Hospital,Guangzhou University of Chinese Medicine,Guangzhou 510168,China;College of Traditional Chinese Medicine,Southern Medical University,Guangzhou 510515,China;The Second Affiliated Hospital of Guangzhou Medical University,Guangzhou 510260,China)
出处
《中国病理生理杂志》
CAS
CSCD
北大核心
2021年第11期2094-2099,共6页
Chinese Journal of Pathophysiology
基金
国家自然科学基金资助项目(No.81873426
No.81973583)。
关键词
再生障碍性贫血
免疫异常
骨髓造血微环境
造血干细胞
Aplastic anemia
Immune abnormality
Bone marrow hematopoietic microenvironment
Hemato-poietic stem cells
作者简介
通讯作者:肖扬,Tel:020-88653562,E-mail:jdxiao111@163.com。
