摘要
自身免疫性溶血性贫血(AHIA)是由于机体产生抗自身红细胞膜抗原的抗体导致红细胞破坏增加,寿命缩短。据有无病因,分为原发性和继发性,继发者约占50%。据致病抗体最佳活性温度分为温抗体型、冷抗体型和冷温抗体混合型。诊断方面注意查明抗体类型及原发病。治疗方案多为经验性治疗,温抗体型AIHA首选糖皮质激素,有效率可达80%,但易复发,停药后持续缓解率小于20%;二线治疗方案包括脾切除和利妥昔单抗(R)。近年来国外研究表明,R有效率高达70%以上,部分学者认为应作为首选二线治疗方案,甚至用于一线治疗。传统治疗如环孢素、达那唑、环磷酰胺、硫唑嘌呤、人免疫球蛋白、吗替麦考酚酯、促红细胞生成素(EPO)、血浆置换、全血置换等均报道有效;新药研究示阿伦单抗、奥法木单抗有效,但均需要大样本前瞻性随机对照试验提供循证学依据。冷抗体型AIHA对糖皮质激素和脾切除疗效差,R应作为一线治疗方案。
Autoimmune hemolytic anemia（AIHA）is an acquired disorder in which autoantibody directed against self red blood cell（RBC）membrane antigens leading to RBC accelerated destruction.AIAH could be divided into idiopathic or secondary（50%）on the basis of with or without etiology,but also it could be classified as warm,cold or mixed antibody type according to the thermal range of the autoantibody.In the diagnosis of AIHA,the type of the antibody involved and primary or secondary should be confirmed.The first choice in warm AIHA was corticosteroid,which was effective in 80% of patients,but cured in less than 20%.For refractory/relapsed cases,the second-line therapy included splenectomy and rituximab.Recently,rituximab was reported that the overall response rate was more than 70%.Regarding as the preferred the second-line therapy of warm AIHA in some experts＇ opinion,it even could be used upfront in some patients. Other options are cyclosporine, danazol, cyclophosphamide, azathioprine,immunoglobulin,mycophenolate mofetil,erythropoietin（EPO）,plasma-exchange,whole blood exchange transfusion.Alemtuzumab and ofatumumab regarding as new drugs need more prospective and comparative studies.In cold AHIA,rituximab was recommended as the first-line treatment,because splenectomy and corticosteroid were usually ineffective.
出处
《临床荟萃》
CAS
2015年第10期1087-1091,共5页
Clinical Focus
关键词
贫血
溶血性
自身免疫性
诊断
治疗
anemia
hemolytic
autoimmune
diagnosis
treatment
