摘要
目的报道1例重型地中海贫血(TM)患儿行HLA全相合同胞骨髓造血干细胞移植(HSCT)术后早期并发自身免疫性溶血性贫血(AIHA)的临床特点、治疗及随访,提高对该并发症的认识。方法回顾性分析深圳市儿童医院血液肿瘤科1例TM患儿的临床资料,复习国内外相关文献。结果重型β-TM患儿行HLA全相合同胞骨髓HSCT术后15 d出现血红蛋白进行性下降,伴乳酸脱氢酶及间接胆红素升高,间接抗人球蛋白试验阴性,直接抗人球蛋白试验IgG阳性、C3d阴性,应用糖皮质激素联合其他免疫抑制剂的治疗效果明显。结论 HSCT术后并发AIHA的主要发生机制可能有多重因素,加强HSCT术后AIHA发病机制的研究,将有利于该并发症的防治和改善预后。
Objective To report the clinical features, its treatment and follow-up of one patient with autoimmune hemolytic anemia （AIHA） following HLA-identical sibling bone marrow transplantation （BMT） , and enhance the recognition of this rare disease in clinical practice. Methods The clinical features, treatment and follow-up data of a case diagnosed as AIHA in our hospital were analyzed, and related literature was reviewed. Results A 4-year-8-month old boy who had thalassemia major underwent HLA-identical sibling BMT in Shenzhen Children＇s Hospital. We found a progressive decrease in hemoglobin, increase in lactate dehydrogenase and indirect bilirubin, concomitant with negative indirect anti-humdirectly anti-human ball testan ball test and C3d, but positive IgG of directly anti-human ball test within 15 days after transplantation. Combined clucocorticoids with other immunosuppressive agents showed notable therapeutic effect. Conclusion Multiple factors presumably involve in the main mechanism of AIHA after hemopoietic stem cell transplantation. It is suggested that more researches on AIHA after hemopoietic stern cell transplantation will contribute to the prevention of the complication and improve the prognosis.
出处
《中国小儿血液与肿瘤杂志》
CAS
2014年第1期31-34,共4页
Journal of China Pediatric Blood and Cancer
作者简介
通讯作者：文飞球，Email：fwen62@126．com
