Velvet Anju John John, Bhutani Shiv, Papachristos Stavros, Dwivedi Reena, Picton Michael, Augustine Titus, Morton Muir
Department of Renal and Pancreas Transplantation, Division of Surgery, Manchester Royal Infirmary, Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
Department of Renal Medicine and Transplant Nephrology, Manchester Royal Infirmary, Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
Oncotarget. 2019 Jan 11;10(4):437-448. doi: 10.18632/oncotarget.26522.
Central Nervous System (CNS) lymphoma is a rare presentation of post-transplantation lymphoproliferative disorder (PTLD).
This single center retrospective study reviewed presentations, management and outcomes of CNS lymphomas in kidney transplant patients transplanted 1968 to 2015, and reviews relevant current literature.
We identified 5773 adult kidney transplant recipients of who 90 had a PTLD diagnosis confirmed. CNS disease was diagnosed in 6/90 (7%). Median age at presentation was 60 years and time from transplant 4.5 years. Immunosuppression at diagnosis included mycophenolate mofetil and prednisolone without calcineurin inhibitor in 5/6 patients. Histological analysis diagnosed monomorphic disease in 5/6, and one polymorphic case with tissue positive for Epstein-barr virus (EBV) in 5/6 cases. Despite this 2/4 EBV positive cases had no detectable EBV in peripheral blood or CSF at diagnosis. Treatment strategies included reduction in immunosuppression in all, chemotherapy (n=5), radiotherapy (n=3), Cytotoxic T-Lymphocytes and Craniotomy (n=2). Patient survival was 40% at 1 year with CTL treated patients surviving beyond three years from diagnosis.
This study supports observational data suggesting MMF treated patients without CNI may have increased risk of disease. Peripheral blood screening for EBV DNAemia does not seem helpful in early identification of those at risk.
中枢神经系统（CNS）淋巴瘤是移植后淋巴细胞增生性疾病（PTLD）的一种罕见表现形式。
这项单中心回顾性研究回顾了1968年至2015年接受肾移植患者中CNS淋巴瘤的临床表现、治疗及预后情况，并对相关的当前文献进行了综述。
我们确定了5773例成年肾移植受者，其中90例确诊为PTLD。6/90（7%）例被诊断为CNS疾病。发病时的中位年龄为60岁，距移植时间为4.5年。6例患者中有5例在诊断时的免疫抑制方案包括霉酚酸酯和泼尼松龙，未使用钙调神经磷酸酶抑制剂。组织学分析显示6例中有5例为单形性疾病，1例多形性病例中6例中有5例组织中爱泼斯坦-巴尔病毒（EBV）呈阳性。尽管如此，2/4例EBV阳性病例在诊断时外周血或脑脊液中未检测到EBV。治疗策略包括所有患者均减少免疫抑制，化疗（n = 5），放疗（n = 3），细胞毒性T淋巴细胞和开颅手术（n = 2）。接受细胞毒性T淋巴细胞治疗的患者从诊断起存活超过三年，1年时患者生存率为40%。
本研究支持观察性数据，提示接受霉酚酸酯治疗且未使用钙调神经磷酸酶抑制剂的患者可能有更高的患病风险。外周血EBV病毒血症筛查似乎无助于早期识别高危人群。
