儿童造血干细胞移植后淋巴组织增殖性疾病影像学和临床表现

Imaging and clinical features of post transplantation lymphoproliferative disorder after hematopoietic stem cell transplantation in children

目的观察儿童造血干细胞移植(HSCT)后淋巴组织增殖性疾病(PTLD)的影像学和临床表现。方法回顾性分析13例经病理证实或临床诊断的HSCT后PTLD患儿,原发病包括再生障碍性贫血10例,骨髓增生异常综合征、湿疹-血小板减少-免疫缺陷综合征和β地中海贫血各1例。结果13例PTLD患儿均见结内组织受累,均累及颈部淋巴结,其中3例累及腹腔淋巴结、3例累及腭扁桃体、2例累及腺样体;3例结外组织受累,包括中枢神经系统受累2例、胃壁受累1例。按部位观察,13例颈部均受累,3例腹部、2例中枢神经系统受累;移植与诊断PTLD时间间隔42~120天,平均(68±25)天;移植前13例均无EB病毒(EBV)感染,移植后均发生EBV感染;经对症治疗,5例好转出院,8例死亡。结论儿童HSCT后PTLD发病较早、进展迅速、可累及全身多部位,移植后早期即需密切筛查、监测外周血EBV DNA载量,警惕PTLD发生;影像学与临床相结合有助于早期确诊。

Objective To observe the imaging and clinical characteristics of post transplantation lymphoproliferative disorders(PTLD)after hematopoietic stem cell transplantation(HSCT)in children.Methods Data of 13 children with PTLD confirmed pathologically or clinically were retrospectively analyzed.Among 13 cases,there were 10 cases of aplastic anemia,1 case of myelodysplastic syndrome,1 case of Wiskott-Aldrich syndrome and 1 case ofβ-thalassemia.Results Intranodal involvements were detected in all 13 cases of PTLD,including cervical lymph nodes in 13 cases,abdominal lymph nodes and palatine tonsils each in 3 cases and adenoids in 2 cases.Extranodal involvements occurred in 3 cases,including central nervous system involvements in 2 cases and gastric wall involvement in 1 case.For the location,neck involvements were observed in all 13 cases,while abdomen and central nervous system involvements were found in 3 and 2 cases,respectively.The time interval between transplantation and diagnosis of PTLD was 42-120 days,with an average of(68±25)days.Epstein-Barr virus(EBV)infection did not exist before transplantation,but occurred in all 13 patients after transplantation.After symptomatic treatments,5 cases went better and discharged from hospital,whereas 8 cases died.Conclusion PTLD in children after HSCT had early onset and rapid progression,which could involve many parts of the body.Early and closely screening and monitoring DNA load of EBV in peripheral blood were necessary to prevent PTLD after transplantation.The combination of imaging and clinic was helpful to early diagnosis.