Post-transplant Lymphoproliferative Diseases: a Case Report and Discussion of Management
T Chan
Hong Kong J Radiol 2013;16(Suppl):S60-4
Post-transplant lymphoproliferative disorders (PTLD) are lymphoid or plasmacytic proliferations that develop in immunosuppressed individuals, particularly in recipients of solid organs, bone marrow, or stem cell allograft. Although rare in the general population, the prevalence of PTLD is increasing among transplant patients, particularly as transplantations have become more frequent procedures in recent years. PTLD are very closely associated with Epstein-Barr virus infection, with up to 80% of all PTLD cases testing positive for Epstein-Barr virus. Histologically, PTLD have three main subtypes as classified by the World Health Organization: early lesions, polymorphic PTLD and monomorphic PTLD, which can be regarded as lying on a pathological continuum. While there are currently no data from phase III clinical trials to guide optimal treatment of PTLD, evidence from phase I and II studies, retrospective analyses and case series, as well as expert opinion, indicate the effectiveness of immediate reduction in immunosuppression as first-line therapy, particularly in early-stage disease. When reduction in immunosuppression is inadequate, the evidence shows that addition of rituximab and / or chemotherapy is a useful and effective option. Radiation has limited indication in PTLD except in localised disease and for palliation. This article describes a case of PTLD in a female patient with end-stage renal failure due to lupus nephritis, who had previously received a cadaveric renal transplant. Her management is discussed with some reference to the published literature and current opinion.
中文摘要
移植後淋巴增生性疾病：病例報告及治療方法的討論
陳守仁
移植後淋巴增生性疾病（PTLD）是指出現在免疫抑制病人中的淋巴或漿細胞增生，尤見於異體器官、骨髓或幹細胞移植病人身上。雖然在一般人口中罕見，在移植病人中PTLD的流行率正在上升，尤其是近年間移植手術越趨頻密。PTLD與Epstein-Barr（EB）病毒感染密切相關，在所有PTLD個案中有高達八成對EB病毒測試呈陽性。組織學上，世界衛生組織將PTLD分為三個主要亞型：早期病變、多形性PTLD和單形性PTLD。這三個分類可被視為一個連續體。雖然目前尚未有第三期臨床試驗數據作為PTLD理想治療的指引，但第一及第二期研究、回顧分析、病例系列報告及專家意見指出，即時降低免疫抑制作為一線治療具有療效，尤其在疾病早期。在免疫抑制下降不足的情況下， 證據顯示加用利妥昔單抗（rituximab）及／或化療是一個有用而有效的選擇。電療在PTLD中的適應證有限，除非用於局部疾病及紓緩治療。本文報導一個因紅斑狼瘡性腎炎出現末期腎衰竭、曾接受屍腎移植的PTLD女病人個案，並參考過往文獻與專家意見，對個案的治療作出討論。