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23例温冷双抗体型自身免疫性溶血性贫血的临床特征

The clinical features of the autoimmune hemolytic anemia with both warm and cold autoantibodies

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【作者】 邢莉民邵宗鸿刘鸿施均白洁王化泉涂梅峰孙娟

【Author】 XING Li-min, SHAO Zong-hong, LIU Hong, SHI Jun, BAI Jie, WANG Hua-quan, TU Mei-feng, SUN Juan. Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China

【机构】 中国医学科学院、中国协和医科大学血液学研究所、血液病医院中国医学科学院、中国协和医科大学血液学研究所、血液病医院

【摘要】 目的了解近十年温冷双抗体型自身免疫性溶血性贫血(AIHA)患者的临床特征,以加深对该病的认识。方法对1994年1月至2004年4月诊断的23例温冷双抗体型AIHA的临床表现、实验室特征及治疗反应进行回顾性分析。结果近十年温冷双抗体型AIHA在同期AIHA中占 22.1%,较20世纪80年代的17.6%有所增加;原发性病例占73.9%;女性多见;自身抗体的类型以复合型为主,含IgM抗体者多见(56.5%),抗体温幅及补体水平与溶血程度相关;对肾上腺皮质激素合并其它免疫抑制剂反应较好;中位随访时间4个月,复发率为77.8%。结论温冷双抗体型AIHA是一种女性多见、原发性为主、与抗体种类和温幅及补体激活相关、复发率较高的溶血性疾病,联合免疫抑制治疗效果较好。

【Abstract】 Objective To study the clinical characteristics of autoimmune hemolytic anemia( AIHA) with both warm and cold autoantibodies. Methods Clinical and laboratory characteristics of 23 cases of AIHA with both warm and cold autoantibodies admitted to our hospital between January 1994 and April 2004 were analyzed retrospectively. Results In comparison with the AIHA patients with both warm and cold autoantibodies in the 1980s, the present patients showed the following features: The proportion of this kind AIHA in all AIHA patients increased from 17.6% to 22. 1%. There were more females, more primary cases (73.9% ) , more mixed subtypes of autoantibodies and more of IgM(56. 5% ). The hemolysis was related with thermal amplitude of autoantibodies and quantity of complement. The response to cortisone and other im-munosuppressive drugs was good. The relapse rate was 77. 8% in a median follow-up time of 4 months. Conclusions AIHA with both warm and cold autoantibodies is related with the type and thermal amplitude of the autoantibody and the activation of complement. It can be treated effectively with combined immunosuppressive therapy, but the relapse rate is high.

  • 【文献出处】 中华血液学杂志 ,Chinese Journal of Hematology , 编辑部邮箱 ,2006年01期
  • 【分类号】R556
  • 【被引频次】11
  • 【下载频次】171
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