重型地中海贫血移植后并发自身免疫性溶血性贫血临床分析

Clinical analysis of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation in thalassemia major

目的探讨重型地中海贫血（地贫）患者异基因造血干细胞移植（allo-HSCT）后并发自身免疫性溶血性贫血（AIHA）诊断、治疗及转归，以提高地贫患者造血干细胞移植疗效。方法回顾性分析2007年7月至2017年12月共计291例行allo—HSCT重型地贫患者的临床资料。结果重型地贫allo—HSCT后AIHA发生率为1．72％（5／291）；AIHA中位发生时间为移植后7（5～12）个月，5例移植后AIHA患者直接和间接Coombs试验均阳性，患者主要表现为头晕、乏力、面色苍白、皮肤巩膜黄染、酱油色尿。228例HLA相合同胞供者移植患者有1例（0．43％）移植后发生AIHA，而63例非亲缘供者移植患者有4例（6．36％）移植后发生AIHA。非亲缘供者移植患者AIHA发生率高于HLA相合同胞供者移植患者。1例患者单用泼尼松治疗死亡，4例患者采用甲泼尼龙联合利妥昔单抗治疗有效，目前生存良好，其中2例Coombs试验转阴。结论该组重型地贫患者allo—HSCT后AIHA发生率为1．72％，Coombs试验有助于诊断移植后AIHA，非亲缘供者移植病例移植后AIHA发生率高于HLA相合同胞供者移植组，利妥昔单抗联合糖皮质激素是治疗重型地贫allo-HSCT后AIHA的有效方法。

Objective To explore the diagnosis, treatment and prognosis of autoimmune hemolytic anemia （AIHA） after allo-HSCT in patients with thalassemia major （TM）. Methods A retrospective analysis of AIHA status after allo-HSCT in 291 TM patients from July 2007 to December 2017 was conducted. Results Five of the 291 TM patients （1.72%） were diagnosed with post-transplant AIHA. The median time of AIHA was 7 （5- 12） months after HSCT. All post-transplant AIHA patients were positive in direct and indirect Coombs test, the main clinical manifestations were dizziness, fatigue, pale complexion, skin and sclera yellow, and soy sauce urine. The incidence of AIHA was higher after unrelated donor transplantation （6.36%, 4/63） compared with that of sibling donor transplantation （0.43%, 1/228）. One patient who received only prednison was dead. Four patients who received rituximab combined with prednisolone were alive, Coombs test in two of them were negative. Conclusions AIHA after allo- HSCT developed in 1.72% patients with TM. Monitoring of Coombs test was important for diagnosis of post-transplant AIHA. The incidence of post-transplant AIHA was higher in unrelated donors compared with that of sibling donors transplantation. Treatment of rituximab combined glucocorticoid was effective strategy for post-transplant AIHA.