儿童肝移植术后淋巴组织增殖性疾病11例临床分析  

Clinical analysis of posttransplant lymphoproliferative disorder in pediatric liver transplant recipients: a report of 11 cases

作　　者：李玉川[1] 沈丛欢 冯佳燕 王建设[1] 谢新宝[1] Li Yuchuan;Shen Conghuan;Feng Jiayan;Wang Jianshe;Xie Xinbao(Department of Hepatology,Children's Hospital of Fudan University,Shanghai 201102,China;Department of General Surgery,Liver Transplantation Center,Affiliated Huashan Hospital,Institute of Organ Transplantation,Fudan University,Shanghai 200040,China;Department of Pathology,Children's Hospital of Fudan University,Shanghai 201102,China)

机构地区：[1]复旦大学附属儿科医院肝病中心,上海201102 [2]复旦大学附属华山医院普外科肝移植中心,复旦大学器官移植研究所,上海200040 [3]复旦大学附属儿科医院病理科,上海201102

出　　处：《中华器官移植杂志》2022年第5期292-297,共6页Chinese Journal of Organ Transplantation

摘　　要：目的探讨儿童肝移植术后淋巴组织增殖性疾病(posttransplant lymphoproliferative disorder,PTLD)的临床表现、病理特征、治疗方案及预后。方法对2016年10月至2021年10月复旦大学附属儿科医院收治的11例儿童肝移植术后PTLD的临床资料进行回顾性分析。其中,男5例,女6例;年龄1~8岁。行亲属活体肝移植术9例,接受死亡器官捐献供肝移植术2例,术后均采用他克莫司联合甲泼尼龙的免疫抑制方案。11例患儿临床表现主要包括:淋巴结肿大,脾大,贫血,发热,消化系统症状(腹泻、腹痛、腹水、便血、肠套叠等);实验室检查方面主要表现为低蛋白血症和转氨酶升高,11例患儿血液EB病毒DNA(EBV-DNA)均阳性;9例患儿行PET-CT检查考虑PTLD;10例患儿根据病理检查结果明确诊断,其中淋巴组织增生3例,浆细胞增生性PTLD 1例,多形性PTLD 2例,弥漫性大B细胞淋巴瘤2例,传染性单核细胞增生性PTLD 1例,Burkitt淋巴瘤1例。结果11例儿童受者确诊PTLD后,均减量或停用他克莫司,11例患儿均接受利妥昔单抗治疗,2例患儿接受化疗(R-COP,R-CHOP方案),2例局部占位病变患儿行手术治疗。截至2022年2月,10例患儿病情好转,1例患儿因感染死亡。结论PTLD是儿童肝移植术后严重的并发症,临床表现多样,早期诊断困难,肝移植术后应密切监测EBV-DNA变化,影像学检查和组织病理学检查有助于早期诊断,以免疫抑制剂减量、利妥昔单抗为基础的治疗方案可改善儿童肝移植术后PTLD的预后。Objective To explore the clinicopathological characteristics,treatments and outcomes of posttransplant lymphoproliferative disorder(PTLD)in pediatric liver transplant recipients.Methods From October 2016 to October 2021,retrospective data analysis was performed for 11 pediatric liver transplant recipients with PTLD.There were 5 males and 6 females with a diagnostic age of 1-8 years.Living donor liver transplantation(LDLT,n=9)and deceased donor liver transplantation(DDLT,n=2)were performed.All recipients received tacrolimus plus methylprednisolone.The major clinical manifestations included lymphadenopathy,splenomegaly,anemia,fever and digestive system symptoms(diarrhea,abdominal pain,ascites,hematochezia&intussusception,etc.).Laboratory tests hinted at hypoproteinemia,elevated transaminases and serum positivity of EBV-DNA.Positron emission tomography and computed tomography(PET-CT)revealed PTLD(n=9).Ten children were diagnosed by pathology,including lymphoid hyperplasia(n=3),plasmacytic hyperplasia PTLD(n=1),polymorphic PTLD(n=2),diffuse large B-cell lymphoma(n=2),infectious mononucleosis PTLD(n=1)and Burkitt lymphoma(n=1).Results After a definite diagnosis of PTLD,tacrolimus was tapered or discontinued.And rituximab was prescribed.Two patients received chemotherapy(R-COP&R-CHOP)while 2 cases of local masses were operated.Up until February 2022,10 cases survived and their conditions improved.One patient died of infection.Conclusions PTLD is one of the most serious and fatal complications after liver transplantation in children.Clinical manifestations are diverse and an early diagnosis is difficult.The changes of EBV-DNA load should be closely monitored after liver transplantation.Imaging and pathological examinations may aid in an early diagnosis of PTLD.A treatment regimen based on immunosuppression reduction and rituximab improves the prognosis of PTLD in pediatric liver transplant recipients.

关 键 词：儿童 肝移植 淋巴组织增殖性疾病 EB病毒 

分 类 号：R726.5[医药卫生—儿科]