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自身免疫性溶血性贫血(AIHA)分为温抗体型和冷抗体型。温抗体型AIHA有原发性和继发性2类,其发病机制为基因突变、免疫功能紊乱、血细胞膜抗原性改变,刺激机体产生相应的红细胞自身抗体或交叉反应抗体,诊断主要依据抗人球蛋白(Coombs)试验,治疗首选肾上腺皮质激素,可用大剂量丙种球蛋白、免疫抑制剂、利妥昔单抗等。冷抗体型AIHA包括冷凝集素综合征(CAS)、阵发性寒冷性血红蛋白尿(PCH)。CAS表现为慢性贫血,逐渐加重,实验室检查有自身凝集反应、血清学筛选试验阳性,轻症不必治疗,重症可用皮质激素、免疫抑制剂等。PCH表现为暴露于寒冷的环境后突然发生大量血管内溶血,可用Donath-Land-steiner进行诊断性试验,一般不需治疗。
Autoimmune hemolytic anemia (AIHA) is divided into warm antibody type and cold antibody type. There are two types of primary and secondary warm AIHA, its pathogenesis is genetic mutation, immune dysfunction, blood cell membrane antigenic changes to stimulate the body to produce the corresponding red blood cell autoantibodies or cross-reactive antibodies, the diagnosis is mainly based on anti-human Coombs test, treatment of choice for adrenocorticotropic hormone, available high-dose gamma globulin, immunosuppressive agents, rituximab and so on. Cold antibody AIHA includes cold agglutinin syndrome (CAS), paroxysmal cold hemoglobinuria (PCH). CAS showed chronic anemia, and gradually increased, laboratory tests have their own agglutination reaction, serological screening test was positive, mild without treatment, severe available corticosteroids, immunosuppressive agents. PCH appears to have a sudden onset of massive intravascular hemolysis after exposure to a cold environment and can be diagnosed using the Donath-Land-steiner, generally without treatment.