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EB病毒（EBV）相关移植后淋巴细胞增殖性疾病（PTLD）是造血干细胞移植后最严重并发症之一。本研究纳入31例再生障碍性贫血（AA）行单倍体移植后发生PTLD的病例，归纳其临床特点，并按照利妥昔单抗治疗1次后是否能下降1个log分为利妥昔单抗单药治疗组或联合治疗组。AA患儿移植后PTLD发生率为10.16%，年龄>10岁者PTLD发生率明显升高（ χ2=11.336， P=0.010）。31例患者中临床诊断27例，病理确诊4例。利妥昔单抗单药治疗组15例，联合治疗组15例。最终27例患儿治愈。3例患儿死亡，2年总生存率为（89.7±5.6）%。标准预处理方案、EBV复阳是影响PTLD预后的危险因素。两种治疗方案对预后影响差异无统计学意义。

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 ( χ2=11.336, P=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.