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目的：临床研究和分析原发性系统性淀粉样变２１例。方法：总结自１９７６年１月至１９９６年９月期间原发性系统性淀粉样变２１例病人的一般情况、临床特点、诊断和治疗。结果：３例伴多发性骨髓瘤，肾脏、心脏和舌多受累，分别占７６％、３３％、３３％，可疑病变器官或组织活检的刚果红染色阳性和／或电镜证实为淀粉样纤维丝沉积，血清有单株免疫球蛋白者５例，尿本周蛋白阳性者４例。结论：原发性系统性淀粉样变临床表现多样，大多为非特异性，易误诊，确诊靠病理活检。
Objective: To study and analyze 21 cases of primary systemic amyloidosis clinically. Methods: To summarize the general situation, clinical features, diagnosis and treatment of 21 patients with primary systemic amyloidosis from January 1976 to September 1996. RESULTS: Three patients with multiple myeloma had multiple kidney, heart and tongue involvement, accounting for 76%, 33% and 33%, respectively. Congo red staining and / or electron microscopy of suspicious lesions in organ or tissue biopsy were confirmed as amyloid fibroids Sedation, serum monoclonal immune globulin in 5 cases, urinary week positive in 4 cases. Conclusion: The clinical manifestations of primary systemic amyloidosis are diverse, mostly non-specific, easily misdiagnosed, confirmed by pathological biopsy.