儿童获得性再生障碍性贫血造血干细胞移植后淋巴组织增生性疾病的临床研究  

Clinical Study of Post-Transplantation Lymphoproliferative Diseases in Children with Acquired Aplastic AnemiaAfter AUogeneic Hematopoietic Stem Cell Transplantation

作　　者：覃霞[1] 陈静[1] 江华[1] 罗长缨[1] 王坚敏[1] 罗成娟[1] 周敏[1] 叶启东[1] 沈树红[1] 李本尚[1] 殷敏智[1] 

机构地区：[1]上海交通大学附属上海儿童医学中心,上海200127

出　　处：《国际输血及血液学杂志》2013年第2期97-101,共5页International Journal of Blood Transfusion and Hematology

摘　　要：目的探讨儿童获得性再生障碍性贫血（AAA）异基因造血干细胞移植（allo-HSCT）后淋巴组织增生性疾病（PTID）的流行病学、临床特点、诊治及预后。方法选择2002年7月至2012年5月于本中心完成allo-HSCT的71例AAA患儿作为研究对象。患儿常规行氟达拉滨＋环磷酰胺＋兔-抗人T淋巴细胞球蛋白±全身放疗为基础的预处理，移植后主要采用环孢霉素和甲氨蝶呤预防移植物抗宿主病。对移植后外周血Epstein-Barr病毒（EBV）-DNA拷贝数持续升高的患儿，抢先予以利妥昔单抗治疗。确诊为PTLD后即予以免疫抑制剂减量或停药。结果AAA患儿行allo-HSCT后PTLD的发生率为4．2％（3／71），临床均表现为抗感染治疗无效的反复发热伴扁桃体和淋巴结肿大。其中，2例患儿移植后予以监测外周血EBV-DNA拷贝数，在PTLD发生前均出现拷贝数的持续升高，且第1次予利妥昔单抗治疗后拷贝数仍持续上升。3例PTLD患儿中，2例治疗有效，随访至今仍无病生存；1例治疗效果不明显，于PTLD发生后34d死亡。结论PTLD是儿童AAAallo-HSCT后一种少见的并发症，病死率较高；动态监测患儿外周血EBV-DNA拷贝数对诊治有一定的指导作用；利妥昔单抗抢先治疗有利于降低PTLD的发生率及病死率。Objective To explore the epidemiology, clinical characteristics, diagnosis, treatment and prognosis of post-transplantation lymphoproliferative diseases （PTLD） in children with acquired aplastic anemia （AAA） after allogeneic hematopoietic stem cell transplantation （allo-HSCT）. Methods From July 2002 to May 2012, a total of 71 children who underwent allo-HSCT with AAA at the Institute of Shanghai Childrenrs Medical Center were included in this study. All of these children＇s clinical data were analyzed. The conditioning regimen included fludarabine, eyclophosphamide and rabbit-antithymocyte globulin with or without total body radiotherapy, and graft-versus-host disease prophylaxis mainly consisted of cyclosporine and methotrexate. For these patients who were monitored by Epstein-Barr virus （EBV）-DNA copies in peripheral blood mononuclear cells after allo-HSCT, a preemptive treatment with anti-CD20 monoclonal antibody rituximab was given when EBV-DNA copies rose persistently. Once PTLD was diagnosed, reduction or withdrawal of immunosuppression was administered. Results The incidence of PTLD for children with AAA after allo-HSCT was 4. 2% （3/71）. All the PTLD patients were presented with persistent fever with no reaction to any antibiotics, progressive antiadoncus and lymphadenectasis. Two cases of the PTLD patients were monitored by EBV-DNA copies after allo-HSCT, and both had persistent high level of EBV-DNA copies before the diagnosis of PTLD, also after the first course of rituximab. After therapies, two of the three PTLD patients were cured and alive till now; however, another one had no effectto the therapy and died 34 days after the diagnosis of PTLD. Conclusions PTLD is a rare and potentially fatal complication for children with AAA after allo-HSCT. Dynamic monitoring of EBV-DNA copies could have certain guiding on the early diagnosis and treatment for PTLD. The preemptive treatment with rituximab could reduce the incidence and mortality of PTLD.

关 键 词：移植后淋巴组织增生性疾病 获得性再生障碍性贫血 异基因造血干细胞移植 儿童 Epstein—Barr病毒 利妥昔单抗 

分 类 号：R72[医药卫生—儿科]