造血干细胞移植治疗原发性免疫缺陷病

摘要

Primary immunodeficiency diseases are a group of inherited disorders with genetic defects in immune cells or molecules, which result in weakened immunity.Hematopoietic stem cell transplantation (HSCT) is an effective therapy currently.There are different sources of HSCT,which have different charac-teristics in the aspect of therapeutic effect and prognosis .Here is to make a review of the progress of HSCT treatment for the four primary immunodeficiency diseases of severe combined immunodeficiency disease,Wis-kott-Aldridge syndrome,hyper-IgM syndrome,and chronic granulomatous disease.%原发性免疫缺陷病是不同基因缺陷引起免疫细胞或免疫分子缺陷，导致机体免疫功能低下的疾病。造血干细胞移植（ HSCT）是目前治疗原发性免疫缺陷病的有效方法。 HSCT来源不同，疗效、预后存在不同的特点。该文就不同来源HSCT治疗严重联合免疫缺陷病、维斯科特-奥尔德里奇综合征、高IgM综合征、慢性肉芽肿病这四种原发性免疫缺陷病的进展进行综述。