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免疫介导小脑共济失调:移植物抗宿主病的一种未知表现。

Immune Mediated Cerebellar Ataxia: An Unknown Manifestation of Graft-versus-Host Disease.

机构信息

Institute of Hematology, Davidoff Cancer Center, Rabin Medical Center, Petach Tikva, Israel,

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel,

出版信息

Acta Haematol. 2019;141(1):19-22. doi: 10.1159/000494423. Epub 2018 Nov 15.

Abstract

Neurologic complications of allogeneic hematopoietic cell transplantation (allo-HCT) include infections, cerebrovascular events, therapy-induced neurotoxicity, recurrent malignancies, and neurologic manifestations of graft-versus-host disease (GVHD). Anti-glutamic acid decarboxylase (GAD) antibody-associated cerebellar ataxia is a well-established disorder of autoimmune origin, but there are no reports in the literature of its occurrence following allo-HCT. We describe a middle-aged woman with chronic GVHD after allo-HCT who presented with a rapidly progressive cerebellar syndrome. Thorough investigation revealed only cerebellar atrophy on brain imaging and positive anti-GAD65 antibodies in serum and cerebrospinal fluid suggesting the diagnosis of anti-GAD antibody-associated cerebellar ataxia. Despite prompt treatment with high-dose corticosteroids, intravenous immunoglobulins, and rituximab, the patient's condition rapidly deteriorated, and she died 4 months later. This case suggests that anti-GAD antibody-associated cerebellar ataxia may be a rare manifestation of chronic GVHD.

摘要

异基因造血细胞移植(allo-HCT)后的神经并发症包括感染、脑血管事件、治疗诱导的神经毒性、复发性恶性肿瘤和移植物抗宿主病(GVHD)的神经表现。抗谷氨酸脱羧酶(GAD)抗体相关小脑性共济失调是一种已确立的自身免疫源性疾病,但文献中尚无 allo-HCT 后发生该病的报道。我们描述了一例 allo-HCT 后发生慢性 GVHD 的中年女性,其表现为进行性小脑综合征。彻底的调查仅显示脑影像学上存在小脑萎缩,以及血清和脑脊液中存在阳性抗 GAD65 抗体,提示抗 GAD 抗体相关小脑性共济失调的诊断。尽管及时使用大剂量皮质类固醇、静脉注射免疫球蛋白和利妥昔单抗进行治疗,但患者病情迅速恶化,4 个月后死亡。该病例提示,抗 GAD 抗体相关小脑性共济失调可能是慢性 GVHD 的一种罕见表现。

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