儿童肝移植后淋巴组织增生性疾病的影像表现和临床分析

Imaging features and clinical analysis of posttransplantation lymphoproliferative disorders after liver transplantation in children

目的探讨儿童肝移植后淋巴组织增生性疾病(PTLD)的影像学表现和临床特点。方法回顾性分析2017年2月至2020年11月上海交通大学医学院附属上海儿童医学中心17例经病理证实或临床诊断的肝移植后PTLD患儿的影像学和临床资料。观察病变累及的部位、范围、密度/信号/回声等特点。结果肝移植时年龄为8(7,11)个月,14例年龄<1岁。肝移植手术与诊断PTLD的时间间隔为22(10,34)个月,范围3~54个月,间隔<1年者(早发型)6例,≥1年者(晚发型)11例。15例伴有EB病毒感染。12例病理确诊的PTLD中,8例为弥漫大B细胞淋巴瘤,3例为Burkitt淋巴瘤,1例为反应性浆细胞增生。17例PTLD患儿中,8例见淋巴结内组织受累,16例结外组织受累,后者包括腹部受累15例。腹部受累部位包括小肠14例、结肠7例、肠系膜4例、肾脏3例、肝脏2例、腹部淋巴结2例、腹膜1例、胃1例,腹部以外受累部位包括淋巴结7例、肺3例、颅骨1例、脑1例、胸膜1例、胸壁1例、鼻咽部1例。最常见的腹部影像异常是肠壁增厚、偏心性肿块、肠腔扩张,可累及小肠、结肠,前者较多。累及肝脏、肾脏者表现为多发肿块。腹部外PTLD最常见表现为淋巴结增大,见于7例患儿,颈部最多见,表现为淋巴结短径大于10 mm,密度/信号均匀,轻度强化。结论儿童肝移植术后数月到数年均可发生PTLD,可累及全身多部位,结外病变多于结内病变,腹部受累最多见,EB病毒感染率高。结合病史、EB病毒感染状态、影像学检查,有助于早期诊断。

Objective To investigate the imaging and clinical characteristics of posttransplantation lymphoproliferative disorders(PTLD)after liver transplantation in children.Methods From February 2017 to November 2020,the imaging and clinical data of 17 children with PTLD after liver transplantation confirmed by pathology or clinical diagnosis were retrospectively analyzed in Shanghai Children′s Medical Center,School of Medicine,Shanghai Jiao Tong University.The site,range,density/signal/echo of the lesions were observed.Results The mean age at transplantation was 8(7,11)months,and 14 patients were younger than 1 year old.The interval between liver transplantation and PTLD diagnosis was 22(10,34)months,ranging from 3 to 54 months.The interval was less than 1 year in 6 patients(early onset)and equal or greater than 1 year in 11 patients(late onset).Fifteen patients had Epstein-Barr virus infection.Among the 12 pathologically confirmed PTLD cases,8 cases were diffuse large B-cell lymphoma,3 cases were Burkitt lymphoma,and 1 case was reactive plasma cell hyperplasia.Among the 17 children with PTLD,8 cases demonstrated involvement of lymph nodes and 16 cases had extranodal involvement.The latter included 15 cases of abdominal involvement.Abdominal sites involved included small intestine in 14 cases,colon in 7 cases,mesentery in 4 cases,kidney in 3 cases,liver in 2 cases,abdominal lymph nodes in 2 cases,peritoneum in 1 case,and stomach in 1 case.The sites of extra-abdominal involvement included lymph nodes in 7 cases,lung in 3 cases,skull in 1 case,brain in 1 case,pleura in 1 case,chest wall in 1 case,and nasopharynx in 1 case.The most common abdominal imaging abnormalities were thickening of the intestinal wall,eccentric mass and dilation of the lumen.Both small intestines and colons could be involved,and the former more commonly.Multiple masses were found in patients with liver and kidney involvement.The most common imaging manifestation of PTLD outside the abdomen was lymph node enlargement,which was found in 7 cases,and the most common was in the neck.The manifestation was shorter diameter of lymph nodes>10 mm,uniform density and signal,with mild enhancement.Conclusions PTLD can occur months to years after liver transplantation in children,which can affect many parts of the whole body.Extranodal lesions are more than intranodal lesions.Abdominal involvement is most common in PTLD,and the infection rate of EB virus is high.Combined with medical history,EB virus infection status and imaging examination are helpful for early diagnosis.