造血干细胞移植后淋巴增殖性疾病临床处理

Treatment of post-transplant lymphoproliferative diseases

移植后淋巴增殖性疾病（PTLD）是造血干细胞移植（HSCT）或实体器官移植受者在移植后发生的一组异质性淋巴系统恶性疾病。易患危险因素包括病毒感染、严重的免疫抑制状态（如去T细胞移植、特异性抗淋巴细胞抗体的使用、原发性免疫缺陷病患者的移植等）、受者年龄和种族背景等。PTLD在细胞来源以B细胞为主，多数（55％～65％）PTLD与EB病毒感染有关。PTLD治疗的第一步是减免疫抑制治疗（RI），单纯RI仅适用于轻型的早期病变。而在RI基础上给予rituximab适用于占多数的进展期PTLD；无反应者再给予联合化疗；原发性中枢神经系统（CNS）FFLD建议采用类似治疗CNS淋巴瘤的方案。新的治疗手段还包括过继免疫治疗、免疫调节治疗和靶点治疗等。

Post-transplant lymphoproliferative diseases （PTLD） are heterogeneous lymphoid disorders that may occur in re- cipients of hematopoietic stem cell transplant or solid organ transplants. Risk factors for FFLD include viral infections, de- gree of immunosuppression （ e. g. ,T cell depletion transplantation, using of anti-lymphocyte antibodies, and patient with pri- mary immunodeficiency disease） ,recipient age and race,and host genetic variations. Most PTLD cases are of B-cell type, while 55% - 65% are EBV-positive. For treatment, reduction of immunosuppression （RI） remains a mainstay, although it alone should be recommended only for some ＂early＂ PTLD cases. For further aggressive PTLD, rituximab treatment together with RI can be used for most cases, with combination chemotherapy reserved for non-responding cases. Primary central nerv- ous system （CNS） FFLD should be treated using similar therapeutic paradigms as for immunocompetent CNS lymphoma. Fi- nally,novel treatment approaches such as adoptive immunotherapy and other rational targeted therapeutics should continue to be explored.