以慢性腹泻起病的原发性系统性淀粉样变性一例并文献复习  

Primary systemic amyloidosis with chronic diarrhea onset:report of one case and review of literature

作　　者：张冉[1] 王全顺[1] 赵瑜[1] 朱海燕[1] Zhang Ran;Wang Quanshun;Zhao Yu;Zhu Haiyan(Department of Hematology,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院血液科,北京100853

出　　处：《白血病．淋巴瘤》2018年第11期660-663,669,共5页Journal of Leukemia & Lymphoma

摘　　要：目的探讨以慢性腹泻起病的原发性系统性淀粉样变性(AL)患者的临床特点、诊断及治疗方法。方法分析2017年11月于解放军总医院就诊的1例以慢性腹泻起病的AL患者的诊疗经过,并进行相关文献复习。结果该例以慢性腹泻起病的患者明确诊断为AL,经过VCD方案治疗病情明显好转,达到完全缓解。结论以慢性腹泻起病的AL需采用多种检测方法以明确诊断及鉴别诊断,以硼替佐米为主的治疗方案可以获得较好的疗效。Objective To explore the clinical features,diagnosis and treatment of chronic diarrhea with primary systemic amyloidosis (AL).Methods The clinical experience of an AL patient with chronic diarrhea who was admitted to chinese PLA General Hospital in November 2017 was presented,and the related literature was reviewed.Results The patient was definitely diagnosed as AL with chronic diarrhea onset; after treatment with VCD regimen,the condition was improved obviously and achieved complete remission. Conclusion AL patient with chronic diarrhea onset needs to undergo various detections to make a clear diagnosis and differential diagnosis,and the bortezomib-based treatment scheme can achieve good curative effect.

关 键 词：淀粉样变性 腹泻 免疫球蛋白轻链 硼替佐米 造血干细胞移植 

分 类 号：R597.2[医药卫生—内科学]