Holley Nolan, Waris Shanawar, Elzahrany Huda, Howell David, Randall Cara, Saifuddin Adnan, Safi Salah Ud Din
Department of Medicine, West Virginia University, Morgantown, WV 26506, USA.
Department of Pathology, Anatomy and Laboratory Medicine, West Virginia University, Morgantown, WV 26506, USA.
J Med Cases. 2025 Jun 9;16(6):195-200. doi: 10.14740/jmc5130. eCollection 2025 Jun.
Primary central nervous system T-cell lymphoma (PCNSTL) is an exceptionally rare subtype of non-Hodgkin lymphoma, comprising only 2% of primary CNS lymphoma cases. Due to its rarity, PCNSTL is often misdiagnosed, lacks standardized treatment guidelines, and carries a poor prognosis. We present a unique case of a 59-year-old-woman with a history of hypertension and hyperlipidemia who initially presented with sudden-onset aphasia and right-sided weakness. Suspected initially of having a cerebrovascular accident (CVA), she received tenecteplase and underwent investigation of CVA, which was largely unremarkable. Six months later, she returned with progressive nausea, vomiting, confusion, and word-finding difficulties. A magnetic resonance imaging (MRI) of the brain showed lesions in the left cerebellum and frontal lobe with vasogenic edema. A suboccipital craniotomy and biopsy confirmed anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma. Positron emission tomography-computed tomography (PET-CT) showed no systemic disease and cerebrospinal fluid (CSF) analysis showed lymphomatous involvement. The patient was initiated on six cycles of methotrexate, cytarabine, and thiotepa (MATRix regimen without rituximab), followed by high-dose chemotherapy (HDC) with carmustine and thiotepa and autologous stem cell transplantation (ASCT). She tolerated treatment and transplant without complications and remains in complete remission 18 months post-transplant. To our knowledge, this is the first reported case of PCNSTL treated successfully with MATRix followed by HDC-ASCT. This case highlights the importance of considering rare CNS lymphomas in patients with atypical neurologic presentations and suggests the use of HDC-ASCT as a promising approach in a disease with no established standard of care.
原发性中枢神经系统T细胞淋巴瘤（PCNSTL）是一种极为罕见的非霍奇金淋巴瘤亚型，仅占原发性中枢神经系统淋巴瘤病例的2%。由于其罕见性，PCNSTL常被误诊，缺乏标准化治疗指南，且预后较差。我们报告了一例独特的病例，一名59岁女性，有高血压和高脂血症病史，最初表现为突发失语和右侧肢体无力。最初怀疑患有脑血管意外（CVA），她接受了替奈普酶治疗并接受了CVA相关检查，结果基本正常。六个月后，她因进行性恶心、呕吐、意识模糊和找词困难再次就诊。脑部磁共振成像（MRI）显示左小脑和额叶有病变，并伴有血管源性水肿。枕下开颅手术及活检证实为间变性淋巴瘤激酶（ALK）阴性的间变性大细胞淋巴瘤。正电子发射断层扫描-计算机断层扫描（PET-CT）显示无全身病变，脑脊液（CSF）分析显示有淋巴瘤累及。患者开始接受六个周期的甲氨蝶呤、阿糖胞苷和噻替派（不含利妥昔单抗的MATRix方案）治疗，随后接受卡莫司汀和噻替派的大剂量化疗（HDC）及自体干细胞移植（ASCT）。她耐受了治疗和移植，未出现并发症，移植后18个月仍处于完全缓解状态。据我们所知，这是首例报道的采用MATRix方案后序贯HDC-ASCT成功治疗的PCNSTL病例。该病例突出了对于非典型神经系统表现患者考虑罕见中枢神经系统淋巴瘤的重要性，并提示HDC-ASCT作为一种在尚无既定标准治疗方案的疾病中颇具前景的治疗方法。
