Swinnen L J
Division of Hematology/Oncology, Loyola University Chicago, Cardinal Bernardin Cancer Center, Rm. 245, 2160 S. First Ave., Maywood, IL 60153, USA.
J Natl Cancer Inst Monogr. 2001(28):38-43. doi: 10.1093/oxfordjournals.jncimonographs.a024255.
Post-transplant lymphoproliferative disorders (PTLDs) comprise a histologic spectrum, ranging from hyperplastic-appearing lesions to frank non-Hodgkin's lymphoma or multiple myeloma histology. Multiple clones may coexist, each representing a discrete lymphomagenic event, a situation that is unique to immunodeficiency states. The incidence varies from 1% in renal recipients to 5% in heart recipients, but can be markedly increased by the use of anti-T-cell therapies or by T-cell depletion in bone marrow transplantation. PTLD continues to arise, even many years after transplantation, and late T-cell lymphomas have recently been recognized. Pretransplant Epstein-Barr virus (EBV) seronegativity increases risk to as high as 30%-50%. PTLD has a highly variable clinical picture; certain patterns are, however, seen. Reversibility of PTLD with reduction in immunosuppressives has long been recognized. Predicting reversibility has been difficult. The presence or absence of bcl-6 mutations has recently been identified as being of predictive value. Surgical resection can be curative. Cytotoxics, although problematic, can also be curative. Long-term remission has been achieved with anti CD21 and CD24 antibodies; efficacy has been reported for interferon alfa and for rituximab. In vitro expanded EBV-specific T cells have been effective as treatment and as prophylaxis in the setting of bone marrow transplantation. EBV viral load measured in blood appears to associate with the emergence of PTLD and may facilitate prophylactic studies. PTLD is a model of immunodeficiency-related EBV lymphomagenesis. Pathogenetic, therapeutic, and prophylactic insights gained from the study of PTLD are likely to be applicable to the acquired immunodeficiency syndrome setting.
移植后淋巴组织增生性疾病（PTLD）包括一系列组织学表现，从增生性病变到明显的非霍奇金淋巴瘤或多发性骨髓瘤组织学表现。多个克隆可能共存，每个克隆代表一个离散的致淋巴瘤事件，这种情况在免疫缺陷状态中是独特的。发病率从肾移植受者中的1%到心脏移植受者中的5%不等，但使用抗T细胞疗法或骨髓移植中T细胞清除可使其显著增加。PTLD甚至在移植多年后仍会出现，最近已认识到晚期T细胞淋巴瘤。移植前EB病毒（EBV）血清学阴性会将风险增加至高达30%-50%。PTLD具有高度可变的临床表现；然而，某些模式是可见的。长期以来人们就认识到通过减少免疫抑制剂可使PTLD可逆。预测可逆性一直很困难。最近已确定bcl-6突变的存在与否具有预测价值。手术切除可治愈。细胞毒性药物虽然有问题，但也可治愈。抗CD21和CD24抗体已实现长期缓解；已报道干扰素α和利妥昔单抗有效。体外扩增的EBV特异性T细胞在骨髓移植环境中作为治疗和预防有效。血液中测量的EBV病毒载量似乎与PTLD的出现相关，可能有助于预防性研究。PTLD是免疫缺陷相关EBV致淋巴瘤的一个模型。从PTLD研究中获得的发病机制、治疗和预防方面的见解可能适用于获得性免疫缺陷综合征情况。
