系统性轻链型淀粉样变的治疗进展

Advances in treatment of systemic light chain amyloidosis

系统性轻链型淀粉样变（AL）是1种克隆性免疫球蛋白轻链沉积导致相关组织器官损伤的浆细胞疾病。确诊的AL患者中，自体外周血造血干细胞移植（APBSCT）是该类患者的首选治疗方案，而不符合移植条件的该病患者可通过化疗提高血液学反应率及延长生存期。目前，AL常用的化疗方案包括以硼替佐米为代表的蛋白酶体抑制剂联合方案、以来那度胺为代表的免疫调节剂联合方案，以及以美法仑为代表的烷化剂联合方案等。近年以NEOD001、抗血清淀粉样P物质（sAP）单克隆抗体等为代表的免疫治疗对AL的疗效显著。此外，克拉霉素、多西环素、表没食子儿茶素没食子酸酯（EGCG）等新型药物，对AL患者的临床疗效仍在探索中。笔者拟就AL的治疗进展进行综述。

Systemic light chain amyloidosis （AL） is a kind of plasma disease which leads to tissue and organ dysfunction caused by the deposit of monoclonal immunoglobulin light chain. Autologous peripheral blood stem cell transplantation （APBSCT） is the first line treatment for patients with confirmed AL. For the ones unengaging the transplantation, chemotherapy can improve the response rate and prolong the survival. Current ways of chemotherapy include proteasome inhibitors, immunomodulatory agents and alkylating agents. In recent years, immunotherapy represented by NEOD001 and antibodies to serum amyloid P component （SAP） has showed to be significantly effective. The clinical response of clarithromycin, doxycycline and epigallocatechin gallate （EGCG） is still under exploration. The article reviews the advance in treatment of AL.