Zhang Z M, Lai Y R, Li Q C, Luo L, Liu R R, Shi L L, Liu L J
Department of Hematology, The First Affiliated Hospital of Guangxi Medial University, Nanning 530021, China.
Zhonghua Xue Ye Xue Za Zhi. 2018 Nov 14;39(11):908-911. doi: 10.3760/cma.j.issn.0253-2727.2018.11.007.
To explore the diagnosis, treatment and prognosis of autoimmune hemolytic anemia (AIHA) after allo-HSCT in patients with thalassemia major (TM). A retrospective analysis of AIHA status after allo-HSCT in 291 TM patients from July 2007 to December 2017 was conducted. 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. 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.
探讨重型地中海贫血(TM)患者异基因造血干细胞移植(allo-HSCT)后自身免疫性溶血性贫血(AIHA)的诊断、治疗及预后。对2007年7月至2017年12月期间291例TM患者allo-HSCT后的AIHA情况进行回顾性分析。291例TM患者中有5例(1.72%)被诊断为移植后AIHA。AIHA的中位发生时间为HSCT后7(5-12)个月。所有移植后AIHA患者直接和间接抗人球蛋白试验均为阳性,主要临床表现为头晕、乏力、面色苍白、皮肤及巩膜黄染、酱油色尿。与同胞供者移植(0.43%,1/228)相比,非血缘供者移植后AIHA的发生率更高(6.36%,4/63)。1例仅接受泼尼松治疗的患者死亡。4例接受利妥昔单抗联合泼尼松龙治疗的患者存活,其中2例抗人球蛋白试验转阴。1.72%的TM患者allo-HSCT后发生AIHA。监测抗人球蛋白试验对诊断移植后AIHA很重要。与同胞供者移植相比,非血缘供者移植后AIHA的发生率更高。利妥昔单抗联合糖皮质激素治疗是移植后AIHA的有效策略。