重型再生障碍性贫血细胞免疫功能异常与强化免疫抑制治疗的关系

重型再生障碍性贫血（SAA）患者骨髓造血功能衰竭的发生、发展与细胞免疫紊乱，特别是T细胞数量、功能的 异常密切相关，因此免疫功能异常在SAA的发病机制中起着重要的作用。目前抗胸腺细胞球蛋白（ATG）或抗淋巴细 胞球蛋白（ALG）联合环胞霉素A（CsA）的强化免疫抑制治疗（IST）对改善SAA 预后有显著疗效。IST 能够使 60％～80％的SAA患者得到血液学恢复即是异常免疫反应损伤造血干细胞的最直接证据。然而，由其他非免疫因 素介导或造血干细胞极度耗竭所致的骨髓衰竭，IST就可能无效。因此，在IST前进行疾病评估和疗效预测具有重要 意义，其中细胞免疫功能异常对初治SAA进行IST的疗效具有重要意义。

Relationship between cellular immune dysfunction and intensive immunosuppressive therapy in patients with severe aplastic anemia

The occurrence and development of bone marrow hematopoietic failure in patients with severe aplastic anemia(SAA)is closely related to cellular immune disorder,especially the abnormal number and function of T cells.Therefore,abnormal immune function plays an important role in the pathogenesis of SAA.At present,intensive immunosuppressive therapy(IST)with ATG/ALG combined with CsA has a significant effect on improving the prognosis of SAA.IST can promote hematopoietic recovery in 60%-80%of patients with SAA,which is the most direct evidence that abnormal immune response can damage hematopoietic stem cells.However,if bone marrow failure is caused by other non-immune factors or extreme depletion of hematopoietic stem cells,IST may be ineffective.It is important to evaluate the disease and predict the curative effect before immunotherapy;moreover,the abnormal cellular immune function plays an important role in the therapeutic effect of IST on newly diagnosed SAA.