儿童自身免疫性溶血性贫血68例临床分析

发布时间：2018-08-30 09:21

【摘要】：目的:研究儿童自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)的临床特征、实验室特点及治疗效果,加深对该疾病的认识,为临床诊断、治疗提供参考。方法:共研究68例AIHA患儿(包括Evans综合征20例),所有病例均接受糖皮质激素治疗,部分患儿接受静脉注射免疫球蛋白(intravenous immunoglobulin,IVIG)及免疫抑制剂等治疗,对临床表现、实验室检查结果及治疗转归进行回顾分析。结果:(1)原发性AIHA 27例,占39.7%,继发性AIHA 41例,占60.3%;Evans综合征(ES)20例,占29.4%;小于3岁患儿占63.2%。(2)发病时溶血程度比较:发病年龄≤1岁组血红蛋白水平低于1岁组,复合抗体组低于单一抗体组,含IgM抗体组低于不含Ig M抗体组(P均0.05);比较游离血红蛋白水平:单纯AIHA组与ES组,≤1岁组与1岁组,复合抗体组与单一抗体组,含IgM抗体组与不含Ig M抗体组之间无统计学差异(P均0.05);间接胆红素水平比较:单纯AIHA组高于ES组(P0.05)。(3)治疗时间比较:ES组长于单纯AIHA组(P0.05),≤1岁组与1岁组、单一抗体组与复合抗体组、含IgM组与不含IgM组、应用IVIG组与未用IVIG组之间治疗时间无统计学差异(P0.05)。(4)45例回访资料完整的患儿中,首次发病后应用糖皮质激素治疗均达到缓解,其中29例(64.4%)持续缓解中,16例(35.6%)在首次缓解后复发,复发率31.5%,9例(56.3%)复发后糖皮质激素治疗仍然有效。比较单纯AIHA组与ES组、≤1岁组与1岁组、单一抗体组与复合抗体组、含IgM抗体组与不含IgM抗体组、应用IVIG组与未用IVIG组复发率,均无统计学差异(P0.05)。结论:发病年龄、不同抗体类型及是否为ES可为临床判断病情严重程度及治疗效果提供依据。对于初次发病及复发患儿,糖皮质激素治疗儿童AIHA疗效确切;ES患儿需要更长的治疗时间;静脉注射免疫球蛋白未能改善治疗效果。
[Abstract]:Objective: to study the clinical features, laboratory characteristics and therapeutic effects of autoimmune hemolytic anemia (autoimmune hemolytic anemia,AIHA) in children, and to provide reference for clinical diagnosis and treatment. Methods: 68 cases of AIHA (including 20 cases of Evans syndrome) were studied. All of them were treated with glucocorticoid, and some of them received intravenous immunoglobulin (intravenous immunoglobulin,IVIG) and immunosuppressant. The results of laboratory examination and the outcome of treatment were analyzed retrospectively. Results: (1) there were 27 cases of primary AIHA (39.7%), 41 cases of secondary AIHA (60.33%), 20 cases of (ES) (29.4%), and 63.2% of children younger than 3 years old. (2) the hemolysis degree of the age 鈮，

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