移植后淋巴细胞增殖性疾病诊断进展与治疗规范

Diagnosis progress and treatment standard of post-transplantation lymphoproliferative diseases

移植后淋巴细胞增殖性疾病（PTLD）是造血干细胞移植或实体器官移植后因免疫缺陷发生的淋巴细胞异常增生性疾病,往往预后很差.发病的主要机制与EB病毒（EBV）相关,可以理解为免疫缺陷状态下的免疫监控缺失,导致感染EBV的B细胞异常增生以及转化.临床上有发热、咽炎、淋巴结肿大、肝脾大等非特异性表现,辅助检查多有EBV血症,确诊需取得组织病理.PTLD病理分类包括早期病变、多形性病变、单形性病变和霍奇金淋巴瘤4大类,体现了病理上渐进性的发展过程.移植后EBV感染及EBV相关PTLD的分级管理包括EBV活化的预防、抢先治疗和目标治疗.利妥昔单抗作为B细胞靶向药物在PTLD的防治中得到越来越多的应用.

Post-transplantation lymphoproliferative diseases （PTLD） is a kind of lymphoproliferative disease caused by immune deficiency after hematopoietic stem cell transplantation and solid organ transplantation, and characterized by poor prognosis. Its major pathogenesis is associated with EB virus （EBV）, which means immune monitoring in the condition of immune deficiency generates abnormal hyperplasia and transformation of B-cell infected by EBV. The clinical manifestations include fever, pharyngitis, lymphadenopathy, hepatosplenomegaly and other nonspecific symptoms. Supplementary examination refers to EBV, and definite diagnosis requires histopathology results. Pathological classification of PTLD includes early lesion, polymorphic lesion, monomorphic lesion, and Hodgkin＇s lymphoma. Management of EBV infection and EBV related to PTLD after transplantation is divided into 3 levels： prevention of EBV activation, preemptive treatment and target therapy. Fortunately, rituximab being a B-cell targeted drug has gained an increasing application.