12例儿童肝移植术后淋巴增殖性疾病的诊治经验  

Posttransplant lymphoproliferative disorder in children treatment experience

作　　者：刘颖 孙丽莹[2] 朱志军 魏林 曲伟 曾志贵 Liu Ying;SunLiying;Zhu Zhijun;Wei Lin;QuWeil;Zeng Zhigui(Department of Liver Trans plantation,Beijng Friendship Hospital,Capital Medical Universily,Beijing 100050,China;Department of critical care medicine,Beijng Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院,国家消化系统疾病临床医学研究中心,肝移植中心,100050 [2]首都医科大学附属北京友谊医院重症医学科,国家消化系统疾病临床医学研究中心,100050

出　　处：《中华器官移植杂志》2020年第9期549-553,共5页Chinese Journal of Organ Transplantation

基　　金：首都临床特色应用研究(Z18100001718220)。

摘　　要：目的总结和探讨儿童肝移植术后淋巴增殖性疾病的诊治要点。方法对2013年6月至2019年8月首都医科大学附属北京友谊医院收治的12例肝移植后淋巴增殖性疾病(PTLD)儿童受者的临床资料进行回顾性分析。对发病特点、病理特征、治疗方案及转归情况进行整理分析。结果12例受者中10例原发病为胆道闭锁,1例为隐源性肝硬化,1例为尿素循环障碍。11例行亲属活体肝移植术(LDLT),1例接受心脏死亡器官捐献(DCD)肝移植,术后均采用他克莫司为基础的免疫抑制方案。所有儿童受者均经病理明确诊断,6例为PTLD传单样病变,1例为滤泡增生型PTLD,1例为多形性PTLD.2例为Burkitt淋巴瘤,1例为弥漫大B细胞淋巴瘤,1例为经典霍奇金淋巴瘤。发病时间为术后11.5个月(5~32个月)。临床表现主要包括发热、淋巴结肿大、肝脾肿大、贫血、黄疸、腹泻腹痛、肠梗阻和肠穿孔。12例儿童受者的血液EBV-DNA水平均为阳性。治疗措施主要包括减少或停用免疫抑制剂,11例接受抗CD20单抗(利妥昔单抗)治疗,4例接受了化学药物治疗(R CHOP,ABVD.COPP/ABV方案),1例联合放射治疗。2例儿童受者因肠穿孔行手术治疗。11例经治疗达到完全缓解,好转出院并规律随访。1例因PTLD复发死亡。结论EBVDNA的监测和影像学检查有助于PTLD的早期诊断,根据不同的病理分型采用减低免疫抑制剂、抗CD20单抗、手术、化疗和放疗等治疗可以改善儿童肝移植受者的预后。Objective To explore the clinical and pathological characteristics,treatments and outcomes of posttransplant lymphoproliferative disorder(PTLD)in liver transplant recipients.Methods From June 2013 to August 2019,retrospective data analysis was performed for 12 pediatric liver transplant recipients with PTLD at Bejing Friendship Hospital.Demographic profiles,clinical details,treatment data and outcomes were collected.Results The primary disease was biliary atresia(10 cases),cryptogenic cirrhosis(1 case)and ornithine transcarbamylase deficiency(OTCD,1 case).Eleven.patients underwent living donor liver transplantation(LDLT)and 1 patient liver transplantation from donation after cardiac death(DCD)donors.All recipients received a tacrolimus(Tac)-based immunosuppressive regimen.A definite diagnosis was made by pathology.The specific etiologies were infectious mononucleosis PTLD(6 cases),florid fllicular hyperplasia PTLD(1 case),polymorphic PTLD(1 case),Burkitt's lymphoma(2 cases),diffuse large B cell lymphoma(1 case)and classical Hodgkin's lymphoma-like PTLD(1 case).The median onset time was 11.5(5~32)months post-transplantation.The major clinical manifestations included fever,anemia,diarrhea,lymphadenopathy,hepatosplenomegaly,jaundice,intestinal obstruction and intestinal perforation.All of them had serum positivity of EB DNA.After a definite diagnosis,immunosuppressants were tapered or discontinued.Anti CD20(rituximab,11 cases).combined chemotheraphy(R CHOP,ABVD&COPP/ABV,4 cases)and combined radiotherapy(1 case)were offered,"I wo cases were operated due to intestinal obstruction.Eleven patients achieved complete response and survived at the time of review.One patient died of relapsing PTLD.Conclusions Detecting EB-DNA load and imaging examinations may provide diagnostic clues for PTLD.Diverse treatments include immunosuppression reduction,anti-CD20 antibody,surgery,radiotherapy and chemotherapy.Early diagnosis and treatment are required for rescuing PTLD patients.

关 键 词：肝移植 儿童 淋巴细胞增生性疾病 EB病毒 

分 类 号：R726.5[医药卫生—儿科]