M蛋白相关性杆状体肌病3例及文献回顾  

Three cases of sporadic late-onset nemaline myopathy with monoclonal gammopathy and literature review

作　　者：董文灏 赵冰 李召 张琛 袁成录 DONG Wenhao;ZHAO Bing;LI Zhao;ZHANG Chen;YUAN Chenglu(Department of Hematology,Qilu Hospital(Qingdao),Cheeloo College of Medicine,Shandong University,Qingdao 266035,Shandong,China;Department of Neurology,Qilu Hospital(Qingdao),Cheeloo College of Medicine,Shandong University,Qingdao 266035,Shandong,China)

机构地区：[1]山东大学齐鲁医院(青岛)血液科,山东青岛266035 [2]山东大学齐鲁医院(青岛)神经内科,山东青岛266035

出　　处：《山东大学学报（医学版）》2022年第10期74-81,共8页Journal of Shandong University:Health Sciences

摘　　要：目的对3例怀疑M蛋白相关性杆状体肌病患者进行肌肉病理检查、M蛋白筛查明确诊断,提高对这一罕见疾病的认识并探讨对此疾病的诊疗方案。方法报道3例M蛋白相关性杆状体肌病,结合文献复习对病例特点进行总结。结果3例患者均表现为进行性加重的肌肉无力症状,完善肌肉活检诊断为杆状体肌病,合并M蛋白,给予行自体造血干细胞移植治疗有效。结论散发的晚发型成人杆状体肌病是一种罕见的、亚急性进展的肌病,常合并M蛋白,针对清除M蛋白的治疗是有效的。Objective Three patients suspected of sporadic late-onset nemaline myopathy with monoclonal gammopathy were diagnosed by muscle pathological examination and monoclonal protein screening,so as to improve the understanding of this rare disease and explore its diagnosis and treatment.Methods The characteristics of this disease were summarized and relevant literatures were reviewed.Results All of the 3 patients presented with progressively aggravated muscle weakness,which was diagnosed by muscle biopsy as sporadic late-onset nemaline myopathy with monoclonal gammopathy.Autologous hematopoietic stem cell transplantation was effective.Conclusion Sporadic late-onset nemaline myopathy is a rare,subacute progressive myopathy characterized by proximal muscle weakness and atrophy,often complicated with monoclonal gammopathy.Therapies directed at eradication of M protein are effective.

关 键 词：M蛋白 杆状体肌病 自体造血干细胞移植 

分 类 号：R746[医药卫生—神经病学与精神病学]