婴幼儿肝移植后淋巴组织增生性疾病的诊断及治疗3例报道  

Diagnosis and treatment of post-transplant lymphoproliferative disorder in pediatric liver transplantation： a report of 3 cases

作　　者：沈丛欢[1] 夏强[1] 陈小松[1] 邱必军[1] 周韬[1] 韩龙志[1] 奚志峰[1] 张建军[1] 

机构地区：[1]上海交通大学医学院附属仁济医院肝脏外科,上海200127

出　　处：《中华器官移植杂志》2014年第6期353-356,共4页Chinese Journal of Organ Transplantation

摘　　要：目的总结并探讨婴幼儿肝移植术后淋巴组织增生性疾病（PTLD）的临床特点及防治措施。方法回顾性分析2010年8月至2013年7月间127例婴幼儿肝移植的临床资料。结果127例婴幼儿肝移植受者中，3例术后发生PTLD，发生率为2．36％（3／127）。3例受者发生PTLD时，均有EB病毒（EBV）感染，检测EBVDNA载量分别为1．5×10^6、2．3×10^6和1．2×10^7拷贝／ml，3例受者临床表现多为发热及腹部症状，1例有浅表淋巴结肿大，均经病例检查确诊；采用减少免疫抑制剂用量或停用免疫抑制剂及使用利妥昔单抗后，1例受者治疗无效后因发生多器官功能衰竭而死亡，2例受者治愈。结论PTLD的发生可能与EBV感染和使用免疫抑制有关。早期确诊和及时治疗是提高受者预后的关键。减少或停用免疫抑制剂以及使用利妥昔单抗是治疗PTLD有效方法。Objective To summarize and discuss the clinical characteristics of and the therapeutic strategy for post-transplant lymphoproliferative disorder （PTLD） in pediatric patients. Method The clinical data of 127 pediatric cases who underwent liver transplantation between August 2010 and July 2013 were retrospectively analyzed. Result Among the 127 cases,3 cases were diagnosed as PLTD. All of them were positive for epsteirrbarr virus （EBV） infection,and the virus loads were1.5×10^6、2.3×10^6 and 1.2×10^7 copies/ml,respectively. Fever and abdominal symptoms were the main clinical manifestations observed, and superficial lymphadenectasis was identified in 1 case. The treatment modalities consisted of reduction or withdrawal of the immunosuppressants and the use of rituximab. Finally,2 cases were cured, and 1 case died from multiple organ failure. Conclusion PTLD is rare in the incidence, but its occurrence will result in lethal outcomes. The incidence and severity of PTLD may be related to the EBV irdection and the use of immunosuppressants. It is necessary to reduce the administration of the immunosuppressants for PTLD, moreover, the use of rituximab is effective for the treatment of PTLD. Nevertheless, transplant clinicians must maintain a high vigilance on PTLD in pediatric patients so that a timely treatment can be made to obtain favorable outcomes.

关 键 词：婴幼儿 肝移植 淋巴组织增生性疾病 

分 类 号：R726.5[医药卫生—儿科]