原发性系统性淀粉样变病1例报告并文献复习

Report of A Case of Primary systemic amyloidosis and Literature Review

目的:探讨原发性系统性淀粉样变病的临床特点和发病机制。方法:结合病例复习文献。结果:发病机制不明确,临床上以心、肾、胃肠道、皮肤等多器官受累为特点,确诊需行组织病理学及免疫组化检查。治疗效果差。结论:早期诊断和早期治疗有可能提高生存率。

Objective：To explore the clinical characteristics and pathogenesis of primary systemic amyloidosis. Methods：Case report combined with literature review. Result： Pathogenesis of primary systemic amyloidosis was not clear. It usually involves multiple organs and systems ,such as heart, kidney,alimentary tract, skin, et al. Diagnosis was confirmed by histopathological and immunohistoehemical findings. The therapeutic effect was general poor. Conclusion： The survival rate may be improved by early diagnosis and early therapy.