儿童肝移植术后淋巴组织增殖性疾病的临床及病理特点——单中心回顾性分析

Clinical and pathological characteristics of lymphoproliferative diseases after liver transplantation in children--A single center retrospective analysis

目的分析儿童肝移植术后淋巴组织增殖性疾病(post-transplant lymphoproliferative disorder,PTLD)的临床及病理特点,为诊断及治疗提供参考依据。方法收集2020年5月至2022年5月天津市第一中心医院儿童肝移植科收治的肝移植术后PTLD患者的临床及病理资料。包括性别、年龄、手术方式、术后免疫抑制方案、PTLD治疗方案、预后、临床表现、肝功能、血浆EBV-DNA、影像学检查结果,依据2016年WHO淋巴组织肿瘤分类进行病理学分型及免疫组化染色结果。回顾性分析患者的临床、病理及预后的特点。结果肝移植术后经病理学确诊的PTLD患者8例,其中男性4例,女性4例,年龄为1~4岁。8例患者原发病均为胆道闭锁且均行亲属活体肝移植术。本组病例中,5例出现淋巴结肿大,5例出现消化系统症状(包括腹痛、肠梗阻、腹水、腹胀),4例出现肝功能异常,3例出现发热,1例出现肝、肾功能异常。血浆EBV-DNA平均为46072 copies/ml。本组病例中非破坏性、多形性和单形性分别占12.5%(1/8)、25%(2/8)和62.5%(5/8)。5例单形性PTLD中伯基特(Burkitt)淋巴瘤、弥漫性大B细胞淋巴瘤和成熟T细胞淋巴瘤,分别占60%(3/5)、20%(1/5)和20%(1/8)。患者确诊后均减量或停用他克莫司并联合利妥昔单抗治疗,其中6例接受化疗,2例接受血液透析治疗,2例行局部占位手术切除治疗。8例患者中7例病情缓解,1例死亡。结论PTLD的早期诊断、根据病理分型选择合理的治疗方案有助于提高患者预后,接受化疗的患儿应警惕肿瘤溶解综合征并及时给予积极有效干预。

Objective To analyze the clinical and pathological characteristics of post-transplant lymphoproliferative disorder(PTLD)in children after liver transplantation,and to provide reference for diagnosis and treatment.Methods The clinical and pathological data of PTLD patients after liver transplantation were collected from May 2020 to May 2022 in the Pediatric Liver Transplantation Department of Tianjin First Central Hospital.The gender,age,surgical method,postoperative immunosuppression regimen,PTLD treatment regimen,prognosis,clinical manifestations,liver function,plasma EBV-DNA,and imaging examination results were included.Pathological classification and immunohistochemical staining results were analyzed according to WHO classification of lymphoid tissue tumors in 2016.The clinical,pathological and prognostic features of the patients were analyzed retrospectively.Results There were 8 patients with pathologically diagnosed PTLD after liver transplantation,including 4 males and 4 females,aged 1~4 years.All the 8 patients had biliary atresia as the primary disease and underwent living donor liver transplantation(LDLT).In this group,5 cases had lymph node enlargement,5 cases had digestive system symptoms(including abdominal pain,intestinal obstruction,ascites,and abdominal distension),4 cases had liver dysfunction,3 cases had fever,and 1 case had abnormal liver and kidney function.The mean plasma EBV-DNA was 46072copies/ml.The non-destructive,pleomorphic and monomorphic cases accounted for 12.5%(1/8),25%(2/8)and 62.5%(5/8),respectively.Burkitt lymphoma,diffuse large B-cell lymphoma and mature T-cell lymphoma accounted for 60%(3/5),20%(1/5)and 20%(1/8)of monomorphic PTLD,respectively.After diagnosis,tacrolimus was tapered or discontinued.Six patients received chemotherapy and 2 patients received hemodialysis.Two cases of local space occupying operation was performed.Of the 8 patients,7 cases had remission and 1 died.Conclusion The early diagnosis of PTLD and the selection of reasonable treatment plan according to pathological classification can improve the prognosis of patients.Children receiving chemotherapy should be alert to tumolysis syndrome and be given active and effective intervention in time.