Chiriac Radu, Baseggio Lucile
Hematology Laboratory, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Lyon, France.
Leuk Res Rep. 2025 Jun 23;24:100524. doi: 10.1016/j.lrr.2025.100524. eCollection 2025.
CNS involvement in T-cell lymphoma is rare, with a 2-6 % risk of relapse. This report presents four cases of CNS relapse in aggressive T-cell lymphomas, including PTCL NOS, AITL, ALCL ALK (-), and ENKTCL. Patients experienced severe neurological symptoms, elevated CSF WBC counts, and resistance to various treatment regimens, including intrathecal HD MTX, salvage chemotherapy, and immunotherapy. Median survival was 1.5-3.5 months, highlighting the poor prognosis. The findings highlight the complexities of managing CNS relapse, the evolving understanding of prophylactic strategies, and the potential for innovative, targeted therapeutic combinations to enhance outcomes for these high-risk patients.
中枢神经系统受累于T细胞淋巴瘤较为罕见，复发风险为2%-6%。本报告介绍了4例侵袭性T细胞淋巴瘤中枢神经系统复发的病例，包括外周T细胞淋巴瘤非特指型（PTCL NOS）、血管免疫母细胞性T细胞淋巴瘤（AITL）、间变性大细胞淋巴瘤ALK（-）和鼻型结外NK/T细胞淋巴瘤（ENKTCL）。患者出现严重的神经症状、脑脊液白细胞计数升高，并且对包括鞘内注射大剂量甲氨蝶呤（HD MTX）、挽救性化疗和免疫治疗在内的各种治疗方案均有耐药性。中位生存期为1.5至3.5个月，突出了预后不良。这些发现凸显了管理中枢神经系统复发的复杂性、对预防策略的不断演变的认识，以及创新的靶向治疗组合改善这些高危患者预后的潜力。
