Yokoyama Emi, Hasegawa Yuta, Wakaki Kentaro, Suzuki Touma, Kajikawa Sayaka, Kanaya Minoru, Izumiyama Koh, Saito Makoto, Morioka Masanobu, Nagai Jun, Ichiki Tomoe, Kikuchi Ryo, Okada Satomi, Ohigashi Hiroyuki, Goto Hideki, Onozawa Masahiro, Hashimoto Daigo, Mori Akio, Teshima Takanori, Kondo Takeshi
Blood Disorders Center, Aiiku Hospital, S4W25, Chuo-ku, Sapporo, 064-0804, Japan.
Department of Hematology, Hokakido University Hospital, Sapporo, Japan.
Int J Hematol. 2025 May;121(5):712-717. doi: 10.1007/s12185-025-03936-y. Epub 2025 Jan 31.
Chronic graft-versus-host disease (cGVHD) is a major serious complication after allogeneic stem-cell transplantation (allo-HSCT), and often mimics autoimmune diseases. Central nervous system (CNS) symptoms are rare manifestations of cGVHD, and are difficult to diagnose. CNS manifestations of cGVHD were discussed in the 2020 National Institutes of Health cGVHD Consensus Project as one of the "atypical cGVHD manifestations" with involvement of various organ systems other than classical cGVHD organs. We experienced a case of myelitis after allo-HSCT diagnosed as cGVHD of the CNS. The neurological symptoms progressed after corticosteroid pulse therapy, resulting in severe paralysis and paresthesia of the lower extremities. The clinical course and magnetic resonance imaging findings showed some similarities with multiple sclerosis. We decided to use rituximab after the patient became refractory to corticosteroids because rituximab has been reported to be effective in multiple sclerosis by suppressing B cells on both sides of the blood-brain barrier. Rituximab was effective for the neurologic symptoms in our case. In atypical cGVHD, treatments used in corresponding autoimmune diseases may be reasonable and effective.
慢性移植物抗宿主病(cGVHD)是异基因造血干细胞移植(allo-HSCT)后的一种主要严重并发症,常类似自身免疫性疾病。中枢神经系统(CNS)症状是cGVHD的罕见表现,且难以诊断。2020年美国国立卫生研究院cGVHD共识项目将cGVHD的CNS表现作为“非典型cGVHD表现”之一进行了讨论,其涉及除经典cGVHD器官外的各种器官系统。我们遇到一例allo-HSCT后发生脊髓炎的病例,诊断为CNS的cGVHD。皮质类固醇脉冲治疗后神经症状进展,导致下肢严重瘫痪和感觉异常。临床病程和磁共振成像结果显示与多发性硬化有一些相似之处。在患者对皮质类固醇耐药后,我们决定使用利妥昔单抗,因为据报道利妥昔单抗通过抑制血脑屏障两侧的B细胞对多发性硬化有效。在我们的病例中,利妥昔单抗对神经症状有效。在非典型cGVHD中,用于相应自身免疫性疾病的治疗可能是合理且有效的。