Lyu Hai-Rong, He Xiao-Yuan, Hao Hong-Jun, Lu Wen-Yi, Jin Xin, Zhao Yu-Jiao, Zhao Ming-Feng
Department of Hematology, Tianjin First Central Hospital, School of Medicine, Nankai University, Tianjin 300192, China.
Department of Neurology, Peking University First Hospital, Beijing 100034, China.
World J Clin Cases. 2021 Feb 26;9(6):1359-1366. doi: 10.12998/wjcc.v9.i6.1359.
Central nervous system graft--host disease (CNS-GVHD) is a rare cause of CNS disorders after allogeneic hematopoietic stem cell transplantation. Currently, establishing a diagnosis of CNS-GVHD is challenging because the diagnostic criteria and diagnostic methods are not well defined and many confounding factors need to be ruled out.
Here, we present two patients with CNS-GVHD. Both patients with a history of acute GVHD or chronic GVHD developed neurological symptoms that could not be explained by other causes, and had abnormal cerebrospinal fluid (CSF) studies as determined by CSF and blood immune biomarker examinations, suggestive of suspected CNS-GVHD. Due to the lack of specific magnetic resonance imaging abnormalities and the rapid clinical deterioration of the patients, we did not attempt to perform a brain biopsy, but prompted the initiation of empirical immunosuppressive therapy. In view of the rapid and favorable response to local and systematic immunosuppressive treatment and the aforementioned neurologic manifestations together with CSF abnormalities and other negative findings, a final diagnosis of CNS-GVHD was made.
CSF and blood immune biomarker examinations facilitated the diagnosis of CNS-GVHD, which are particularly suitable for patients who are critically ill and require urgent treatment and for those who are unsuitable for invasive diagnostic procedures.
中枢神经系统移植物抗宿主病(CNS-GVHD)是异基因造血干细胞移植后中枢神经系统疾病的罕见病因。目前,CNS-GVHD的诊断具有挑战性,因为诊断标准和诊断方法尚未明确界定,且需要排除许多混杂因素。
在此,我们报告两例CNS-GVHD患者。两名有急性移植物抗宿主病(GVHD)或慢性GVHD病史的患者出现了无法用其他原因解释的神经症状,并且通过脑脊液(CSF)和血液免疫生物标志物检查确定脑脊液检查异常,提示疑似CNS-GVHD。由于缺乏特异性磁共振成像异常以及患者临床病情迅速恶化,我们未尝试进行脑活检,而是启动了经验性免疫抑制治疗。鉴于对局部和全身免疫抑制治疗的快速且良好反应以及上述神经学表现、脑脊液异常和其他阴性结果,最终诊断为CNS-GVHD。
脑脊液和血液免疫生物标志物检查有助于CNS-GVHD的诊断,特别适用于病情危急且需要紧急治疗的患者以及不适用于侵入性诊断程序的患者。