Matsui Motohiro, Shimizu Mariko, Ioi Aya, Mayumi Azusa, Higuchi Kohei, Sawada Akihisa, Sato Maho, Yasui Masahiro, Yanagihara Keiko, Inoue Masami
Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Department of Pediatrics, National Center for Global Health and Medicine, Tokyo, Japan.
Child Neurol Open. 2016 Apr 4;3:2329048X15620641. doi: 10.1177/2329048X15620641. eCollection 2016 Jan-Dec.
A 16-month-old girl was diagnosed with Epstein-Barr virus hemophagocytic lymphohistiocytosis and transferred to our hospital on the 58th day of the hemophagocytic lymphohistiocytosis after treatment failure according to the Hemophagocytic Lymphohistiocytosis-2004 protocol. On admission to our hospital, she had a flaccid paralysis of her lower limbs. Nerve conduction studies showed a acute motor axonal neuropathy, and a diagnosis of Guillain-Barre syndrome was established. Intravenous immunoglobulin G was started on the 57th day of the Guillain-Barre syndrome. To date, her neurological recovery is incomplete. For hemophagocytic lymphohistiocytosis, after treatment failure of THP-COP regimen (pirarubicin, cyclophosphamide, vincristine, and prednisone) and 2 courses of ESCAP regimen (etoposide, prednisone, cytarabine, L-asparaginase), we are now in the process of coordinating unrelated umbilical cord blood transplantation. To the best of our knowledge, we report the youngest case of Guillain-Barre syndrome accompanied by Epstein-Barr virus hemophagocytic lymphohistiocytosis. Rapid progression of Guillain-Barre syndrome, the electrophysiological subtype of Guillain-Barre syndrome, and treatment delay possibly led to poor neurological outcome.
一名16个月大的女孩被诊断为Epstein-Barr病毒噬血细胞性淋巴组织细胞增生症,在按照噬血细胞性淋巴组织细胞增生症-2004方案治疗失败后的第58天被转至我院。入院时,她下肢弛缓性麻痹。神经传导研究显示为急性运动轴索性神经病,确诊为吉兰-巴雷综合征。在吉兰-巴雷综合征的第57天开始静脉注射免疫球蛋白G。迄今为止,她的神经功能恢复不完全。对于噬血细胞性淋巴组织细胞增生症,在THP-COP方案(吡柔比星、环磷酰胺、长春新碱和泼尼松)及2个疗程的ESCAP方案(依托泊苷、泼尼松、阿糖胞苷、L-天冬酰胺酶)治疗失败后,我们目前正在协调无关脐血移植。据我们所知,我们报告了最年轻的伴有Epstein-Barr病毒噬血细胞性淋巴组织细胞增生症的吉兰-巴雷综合征病例。吉兰-巴雷综合征的快速进展、吉兰-巴雷综合征的电生理亚型以及治疗延迟可能导致了不良的神经功能预后。