Objectives: To determine the effect of glucose-6-phosphate-dehydrogenase (G6PD) deficiency on patients' complications and prognosis following allogeneic stem cell hematopoietic transplantation (allo-HSCT) . Methods: 7 patients with G6PD deficiency (study group) who underwent allo-HSCT at Peking University People's Hospital from March 2015 to January 2021 were selected as the study group, and thirty-five patients who underwent allo-HSCT during the same period but did not have G6PD deficiency were randomly selected as the control group in a 1∶5 ratio. Gender, age, underlying diseases, and donors were balanced between the two groups. Collect clinical data from two patient groups and perform a retrospective nested case-control study. Results: The study group consisted of six male patients and one female patient, with a median age of 37 (range, 2-45) years old. The underlying hematologic diseases included acute myeloid leukemia (n=3), acute lymphocytic leukemia (n=2), and severe aplastic anemia (n=2). All 7 G6PD deficiency patients achieved engraftment of neutrophils within 28 days of allo-HSCT, while the engraftment rate of neutrophils was 94.5% in the control group. The median days of platelet engraftment were 21 (6-64) d and 14 (7-70) d (P=0.113). The incidence rates of secondary poor graft function in the study group and control group were 42.9% (3/7) and 8.6% (3/35), respectively (P=0.036). The CMV infection rates were 71.4% (5/7) and 31.4% (11/35), respectively (P=0.049). The incidence rates of hemorrhagic cystitis were 57.1% (4/7) and 8.6% (3/35), respectively (P=0.005), while the bacterial infection rates were 100% (7/7) and 77.1% (27/35), respectively (P=0.070). The infection rates of EBV were 14.3% (1/7) and 14.3% (5/35), respectively (P=1.000), while the incidence of fungal infection was 14.3% (1/7) and 25.7% (9/35), respectively (P=0.497). The rates of post-transplant lymphoproliferative disease (PTLD) were 0% and 5.7%, respectively (P=0.387) . Conclusions: The findings of this study indicate that blood disease patients with G6PD deficiency can tolerate conventional allo-HSCT pretreatment regimens, and granulocytes and platelets can be implanted successfully. However, after transplantation, patients should exercise caution to avoid viral infection, complications of hemorrhagic cystitis, and secondary poor graft function.
目的: 探讨葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者和非G6PD缺乏症患者在异基因造血干细胞移植(allo-HSCT)过程中预处理毒性、移植并发症和生存的差异。 方法: 收集2015年3月至2021年1月G6PD缺乏症患者在北京大学人民医院行allo-HSCT的连续病例作为研究组。以1∶5的比例随机抽选同期接受allo-HSCT,且性别、年龄、疾病、移植方式匹配的非G6PD缺乏患者作为对照组。收集两组患者的临床资料,进行回顾性巢式病例对照研究。 结果: 共7例G6PD缺乏患者进入研究组,35例非G6PD缺乏患者作为对照组。研究组7例G6PD缺乏患者中男6例,女1例,中位年龄37(2~45)岁,均无明显G6PD缺乏症相关临床症状;血液系统原发病包括急性髓系白血病3例、急性淋巴细胞白血病2例、重型再生障碍性贫血2例。移植后28 d内研究组所有患者均获得粒细胞植入,对照组粒细胞植入率为94.3%;研究组、对照组粒细胞植入中位时间分别为13(11~17)d、12(10~23)d(P=0.601),血小板植入中位时间分别为21(6~64)d、14(7~70)d(P=0.113)。研究组、对照组继发性移植物功能不良(PGF)发生率分别为42.9%(3/7)、8.6%(3/35)(P=0.036),巨细胞病毒(CMV)感染发生率分别为71.4%(5/7)、31.4%(11/35)(P=0.049),出血性膀胱炎发生率分别为57.1%(4/7)、8.6%(3/35)(P=0.005),细菌感染发生率分别为100.0%(7/7)、77.1%(27/35)(P=0.070),EB病毒(EBV)感染发生率分别为14.3%(1/7)、14.3%(5/35)(P=1.000),真菌感染发生率分别为14.3%(1/7)、25.7%(9/35)(P=0.497),移植后淋巴细胞增殖性疾病(PTLD)发生率分别为0%(0/7)、5.7%(2/35)(P=0.387)。 结论: 合并G6PD缺乏的血液病患者可耐受常规allo-HSCT预处理方案,粒细胞、血小板可顺利植入,但移植后需警惕病毒感染、出血性膀胱炎及继发性移植物功能不良。.
Keywords: Allogeneic hematopoietic stem cell transplantation; Complication; G6PD deficiency; Prognosis.