儿童自身免疫性溶血性贫血29例临床特点和治疗分析

Clinical features and treatment of autoimmune hemolytic anemia in 29 children

目的探讨自身免疫性溶血性贫血的病因及治疗。方法回顾分析2013年1月至2015年12月收治的29例自身免疫性溶血性贫血患儿的临床资料。结果 29例患儿中,原发性10例、继发性19例,其中11例发生于感染后。主要临床表现为面色苍白、黄疸、尿色加深及肝脾肿大,21例Coombs试验阳性。29例患儿中,肾上腺皮质激素治疗反应良好22例;治疗效果不佳7例,在联合丙种球蛋白治疗后效果良好。结论自身免疫性溶血性贫血治疗首选药物为肾上腺皮质激素,丙种球蛋白可提高疗效。

Objective To analyze the etiology and treatment of autoimmune hemolytic anemia. Methods The clinical data of 29 children with autoimmune hemolytic anemia during January 2013 to December 2015 were retrospectively analyzed.Results In 29 children, 10 cases were idiopathic 19 cases were secondary and 11 cases occurred after infections. The main clinical manifestations were pallor, jaundice, dark urine, and hepatosplenomegaly. 21 cases were Coombs test positive. In29 children, 22 cases had a good response to adrenocortical hormone therapy while in 7 cases which had not response toadrenocortical hormone, good efficacy was achived after combined with the gamma globulin treatment. Conclusions The first line drug for autoimmune hemolytic anemia treatment is adrenocortical hormone. The gamma globulin can improve the efficacy