A disease warranting attention from neurosurgeons: primary central nervous system post-transplant lymphoproliferative disorder

Lei Jin, Di Lu, Feng Yan, Jinkun Han, Penghu Wei, Yiqiang Zhou, Yaming Wang, Yongzhi Shan, Guoguang Zhao
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Abstract

Primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) is a rare condition, posing diagnostic and treatment challenges, with histological biopsy essential for diagnosis. Standardized treatment protocols are lacking. This disease requires urgent attention due to the increasing number of organ transplant surgeries and the use of immunosuppressive agents.From 2020 to 2023, our center diagnosed five patients with PCNS-PTLD. We reviewed their clinical records and conducted a comprehensive analysis of 22 literatures on PCNS-PTLD cases following renal transplantation or allogeneic hematopoietic stem cell transplantation (HSCT).Four patients had previously received a kidney transplant, one had undergone allogeneic HSCT. The median time from the last transplant surgery to the diagnosis of PCNS-PTLD differs between kidney transplant (21.5 years) and allogeneic HSCT (9 months). Common symptoms included motor weakness (n = 4), headache (n = 2), confusion (n = 2), and nausea (n = 2), with ring-enhancing (n = 5), typically solitary (n = 3) and supratentorial (n = 3) lesions on imaging. Diagnosis involved robot-assisted stereotactic brain biopsy (n = 4) or craniotomy (n = 1), all showing Epstein-Barr virus and CD20 positivity. Most cases (n = 4) were monomorphic diffuse large B-cell lymphoma. Treatment included rituximab (n = 3), surgical resection (n = 2), zanubrutinib (n = 1), whole-brain radiation (n = 1), and methotrexate (n = 1). At the last follow-up, the median duration of follow-up for all patients was 19 months. During this time, 3 patients had died and 2 patients were still alive.In patients with a history of kidney transplantation or allogeneic HSCT who are on long-term immunosuppressive therapy, any neurological symptoms, particularly the presence of supratentorial ring-enhancing masses in the brain on imaging, whether solitary or multiple, should raise high suspicion for this disease, warranting a timely brain biopsy. Additionally, we found that besides reducing immunosuppressants, zanubrutinib may be a potential, safe, and effective treatment for this condition. Moreover, post-surgical administration of rituximab in conjunction with whole-brain radiotherapy also appears to be a potentially safe and effective approach.
值得神经外科医生关注的疾病:原发性中枢神经系统移植后淋巴增生性疾病
原发性中枢神经系统移植后淋巴增生性疾病(PCNS-PTLD)是一种罕见疾病,给诊断和治疗带来了挑战,组织学活检是诊断的关键。目前还缺乏标准化的治疗方案。由于器官移植手术和免疫抑制剂的使用日益增多,这种疾病亟需关注。从2020年到2023年,我们中心共诊断出5例PCNS-PTLD患者。我们回顾了他们的临床记录,并对22篇关于肾移植或异体造血干细胞移植(HSCT)后PCNS-PTLD病例的文献进行了综合分析。从最后一次移植手术到确诊PCNS-PTLD的中位时间,肾移植(21.5年)和异基因造血干细胞移植(9个月)有所不同。常见症状包括运动无力(4例)、头痛(2例)、意识模糊(2例)和恶心(2例),影像学表现为环状增强(5例)、典型单发(3例)和幕上病变(3例)。诊断涉及机器人辅助立体定向脑活检(4 例)或开颅手术(1 例),所有病例均显示 Epstein-Barr 病毒和 CD20 阳性。大多数病例(4 例)为单形弥漫大 B 细胞淋巴瘤。治疗方法包括利妥昔单抗(3例)、手术切除(2例)、扎鲁替尼(1例)、全脑放射(1例)和甲氨蝶呤(1例)。最后一次随访时,所有患者的中位随访时间为 19 个月。对于有肾移植或异体造血干细胞移植史且长期接受免疫抑制治疗的患者,如果出现任何神经系统症状,尤其是影像学检查发现脑室上有环形强化肿块,无论是单发还是多发,都应高度怀疑该病,并及时进行脑活检。此外,我们还发现,除了减少免疫抑制剂的使用,扎鲁替尼可能是治疗这种疾病的一种潜在、安全而有效的方法。此外,手术后使用利妥昔单抗并配合全脑放疗似乎也是一种潜在的安全有效的方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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