恶性血液病患者异基因造血干细胞移植后营养障碍性神经系统疾病临床分析
Clinical Analysis of Ataxia as Major Performance in the Dystrophy Neuropathy Cases Following Allogeneic Hematopoietic Stem Cell Transplantation
-
摘要: 目的 分析恶性血液病患者异基因造血干细胞移植(hematopoietic stem cell transplantation,HSCT)后以共济失调为主要表现的营养障碍性神经系统疾病特点。 方法 回顾性分析自2010年1月1日至2020年1月31日在广东省人民医院血液科接受HSCT治疗,术后诊断为营养障碍性神经系统疾病病例的临床特点、诊疗经过以及预后,并在万方、维普、CNKI及PubMed数据库检索相关文献进行分析,分别以“韦尼克脑病(Wernicke's encephalopathy,WE)”或“亚急性脊髓联合变性(subacute combined degeneration of spinal cord,SACD)”和“异基因造血干细胞移植”或“骨髓移植”或“移植”等关键词进行检索,检索时间截止至2020年1月31日。 结果 482例恶性血液病患者接受HSCT,有5例出现以共济失调为表现的神经系统病变,占1%。其中4例伴有认知异常,结合影像学等资料临床诊断WE,与文献检索到国内外20多例病例临床症状相似,但发现与移植物抗宿主病(graft versus host disease,GVHD)、血栓性微血管病(thrombotic microangiopathy,TMA)有重叠之处,予维生素B1等治疗后一周余好转。1例伴排尿困难、臀部会阴麻木,结合影像学等资料临床诊断SACD,与文献检索到日本的2例病例的临床症状及影像资料相似、且均有大剂量甲氨蝶呤的使用史,但日本这2例病例均告死亡,最终尸检验证影像结果;我们的患者在予甲酰四氢叶酸钙、腺苷蛋氨酸、氨茶碱、维生素B12等治疗后,情况逐渐好转而生存。 结论 营养障碍性疾病是HSCT后容易忽视的并发症,常与GVHD、TMA伴随,缺乏实验室检测手段,死亡率高,但根据临床症状及影像学资料早期诊断、早期干预效果好。对于移植术后出现共济失调的患者,如合并有认知异常或脊髓病的症状,应注意诊断WE或SACD,维生素B1或叶酸、维生素B12等治疗安全有效。Abstract: Objective To analysis the clinical characteristics of dystrophy neuropathy cases after allogeneic hematopoietic stem cell transplantation(HSCT). Methods The clinical charactaristics, diagnostic and therapeutic process and prognostic follow-up in patients diagnosed of dystrophy neuropathy following HSCT between 1st January 2010 to 31st January 2020 at Department of Hematology, Guangdong Provincial People's Hospital were retrospectively analyzed. A systematic literature review was performed for similar published cases in Wanfang, Weipu,CNKI and PubMed database, using keywords as followed: “Wernicke's encephalopathy (WE)” OR “subacute combined degeneration of spinal cord (SACD)”, AND “allogeneic stem cell transplantation” OR“HSCT”OR “bone marrow transplantation” OR “transplantation”, et al. The retrieval time was up to 31st January 2020. Results Five patients were accounted for about 1% of a11 482 patients who received HSCT at that time. Typical symptom was ataxia,four patients accompany with abnormal cognition, similar with the 20 cases in reviewed literatures, but overlap with graft versus host disease (GVHD) and thrombotic microangiopathy (TMA), all were diagnosed as WE and were ameliorated after receiving vitamin B1 supplement therapy. One patient with myelopathy accompany with dysuria and numbness of buttocks and perineum was diagnosed as SACD, symptoms and radiological information were similar with the 2 cases confirmed by autopsy in Japan, all with a history of high doses methotrexate, and the prognosis was good for our patient after treatment of leucovorin, ademetionine, aminophylline and vitaminB12. Conclusions Dystrophy neuropathy was an easily overlooked complication after HSCT, which was often accompanied by GVHD and TMA. It lacked laboratory detection methods and had a high mortality rate. However, early intervention after early diagnosis according to clinical symptoms and imaging data was effective. When the patients appear ataxia with abnormal cognition or myelopathy after HSCT, WE or SACD should be awared, and vitamin B1 or leucovorin, vitaminB12 were safe and effective treatment.