儿童异基因造血干细胞移植(allo-HSCT)后淋巴细胞增殖性疾病(PTLD)的诊治及预后

Diagnosis Treatment and Prognosis of Children with PTLD after Allo-HSCT

目的探讨儿童异基因造血干细胞移植(allogeneic hematopoietic stem cell transplantation,allo-HSCT)后淋巴细胞增殖性疾病(allo-HSCT post-transplantation lymphoproliferative disease,PTLD)的诊治及预后。方法收集接受alloHSCT的患儿200例,其中5例患儿发生PTLD。分析allo-HSCT后EB病毒重激活的危险因素,并结合5例患者的临床资料分析allo-HSCT后PTLD患者的治疗及预后。结果对EB病毒重激活的6种危险因素进行单因素分析发现,性别、血型与EB病毒重激活的发生无相关性(P>0.05),原发病种、HLA匹配度、干细胞来源、GAHD与EB病毒重激活的发生率相关(P<0.05)。多因素分析发现,HLA完全相符的患儿EB病毒重激活的发生率较低(P<0.05),发生GAHD的患者发生EB病毒重激活的概率明显增加(P<0.05)。5例PTLD患者的EBV-DNA载量较高,均在104以上;PTLD患者的平均发病时间为65.6 d。5例患者随访过程中,1例因多脏器功能衰竭而死亡。结论 PTLD的发生与EB病毒的重激活密切相关,可通过降低危险因素的发生率、预防阶段使用利妥昔单抗清除供者和宿主的记忆B细胞等方法,降低EB病毒的重激活,从而减少PTLD的发生。

Objective To explore the diagnosis ,treatment and prognosis of children with PTLD after allo-HSCT.Meth-ods 200 cases treated with allo-HSCT were collected ,of which 5 cases had PTLD.Risk factors of EB virus reactivation after allo-HSCT were analyzed ,and treatment and prognosis of PTLD patients after allo-HSCT were analyzed combined with the clinical da-ta.Results Single factor analysis was performed on 6 of the risk factors of EB virus reactivation.There was no correlation be-tween sex,blood type and EB virus reactivation（P〉0.05）.Incidence of primary disease,HLA matching,stem cell source,GAHD and EB virus reactivation were correlated （P〈0.05）.Multivariate analysis revealed that HLA was completely consistent with the incidence of viral reactivation of EB virus （P〈0.05）,and the probability of occurrence of EB virus reactivation in patients with GAHD was significantly increased（P〈0.05）.EBV-DNA load of 5 patients with PTLD was higher than 104,and the average time of onset was 65.6 days.5 cases were followed up,1 cases died due to multiple organ failure.Conclusion The occurrence of PTLD is closely related to the reactivation of EB virus.By reducing the incidence of risk factors ,prevention phase can be used to remove the donor and host memory B cells and other methods to reduce the incidence of EB virus reactivation ,thereby reducing the incidence of PTLD.