Shimada Tomohito, Bando Kana, Takahata Atsushi, Toyota Shigeo
Department of Hematology, Yokosuka Kyosai Hospital, 1-16 Yonegahamadori, Yokosuka, Kanagawa, Japan.
Ann Hematol. 2024 Dec;103(12):5951-5955. doi: 10.1007/s00277-024-06029-8. Epub 2024 Oct 1.
Primary leptomeningeal malignant lymphoma (PLML) is a rare subtype of primary central nervous system lymphoma (PCNSL). Treatment is often based on PCNSL, but currently there is no established treatment strategy due to its rarity. We report a case of a 46-year-old male diagnosed with PLML through cerebrospinal fluid cytology and flow cytometry, presenting with multiple cranial nerve palsies and L5 radiculopathy. The patient achieved complete remission (CR) with R-MPV (rituximab, methotrexate, procarbazine, and vincristine) combined with intrathecal chemotherapy (methotrexate, cytarabine, and prednisolone). This was followed by autologous stem-cell transplantation (ASCT) using a thiotepa-based conditioning regimen, resulting in sustained CR. A literature review on the use of ASCT for PLML revealed three reported cases, including ours, all achieving CR with ASCT and minimal adverse events. These findings suggest that ASCT can be a promising consolidation therapy for PLML. Further studies are needed to establish standardized treatment protocols for this rare condition.
原发性软脑膜恶性淋巴瘤(PLML)是原发性中枢神经系统淋巴瘤(PCNSL)的一种罕见亚型。治疗通常基于PCNSL,但由于其罕见性,目前尚无既定的治疗策略。我们报告一例46岁男性,通过脑脊液细胞学和流式细胞术诊断为PLML,表现为多发性颅神经麻痹和L5神经根病。该患者采用R-MPV(利妥昔单抗、甲氨蝶呤、丙卡巴肼和长春新碱)联合鞘内化疗(甲氨蝶呤、阿糖胞苷和泼尼松龙)实现了完全缓解(CR)。随后采用基于噻替派的预处理方案进行自体干细胞移植(ASCT),实现了持续CR。对ASCT用于PLML的文献综述显示,包括我们的病例在内共报告了3例,所有病例通过ASCT均实现了CR且不良事件最少。这些发现表明,ASCT可能是PLML一种有前景的巩固治疗方法。需要进一步研究来为这种罕见疾病建立标准化治疗方案。