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[单倍体相合移植后再生障碍性贫血患儿中基于利妥昔单抗治疗的爱泼斯坦-巴尔病毒相关淋巴细胞增殖性疾病:一项前瞻性单中心研究]

[Rituximab based treatment in pediatric Epsstain Bar Virus associated lymphocyte proliferative diseases after aplastic anemia with haplo-identical transplantation:a prospective single centre study].

作者信息

Zhang F, Hu G H, Suo P, Xu Z L, Bai L, Wang H F, Huang S Y M, Xu L P, Chang Y J, Zhang X H, Huang X J, Cheng Y F

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2024 Jul 14;45(7):678-682. doi: 10.3760/cma.j.cn121090-20231229-00345.

Abstract

Epstein-Barr virus (EBV) associated post-transplant lymphoproliferative disorders (PTLD) are one of the most severe complications after hematopoietic stem cell transplantation (HSCT). This study includes 31 cases of aplastic anemia (AA) patients who developed PTLD after haploidentical transplantation, summarizing their clinical characteristics and categorizing them into either rituximab monotherapy group or combination therapy group based on whether their condition improved by 1 log after a single dose of rituximab. The incidence of PTLD after HSCT in children with AA was 10.16%, and the incidence of PTLD in patients with age >10 years was significantly increased ((2)=11.336, =0.010). Of the 31 patients, 27 were clinically diagnosed and 4 were pathologically confirmed. Finally, 15 patients were classified into the rituximab treatment group and 15 patients into the combination treatment groups. Finally three patients died, and the 2-year overall survival rate was (89.7±5.6) %. Standard pre-treatment protocols and EBV reactivation are risk factors affecting the prognosis of PTLD. There was no statistically significant difference in the impact of the two treatment schemes on prognosis.

摘要

爱泼斯坦-巴尔病毒(EBV)相关的移植后淋巴组织增生性疾病(PTLD)是造血干细胞移植(HSCT)后最严重的并发症之一。本研究纳入了31例单倍体移植后发生PTLD的再生障碍性贫血(AA)患者,总结其临床特征,并根据单剂量利妥昔单抗治疗后病情是否改善1个对数将其分为利妥昔单抗单药治疗组或联合治疗组。AA患儿HSCT后PTLD的发生率为10.16%,年龄>10岁患者的PTLD发生率显著升高(χ²=11.336,P=0.010)。31例患者中,27例为临床诊断,4例为病理确诊。最终,15例患者被分为利妥昔单抗治疗组,15例患者被分为联合治疗组。最终3例患者死亡,2年总生存率为(89.7±5.6)%。标准预处理方案和EBV再激活是影响PTLD预后的危险因素。两种治疗方案对预后的影响无统计学差异。

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