继发性淀粉样变的临床特点

Clinical features of secondary amyloidosis with a report of 8 cases

目的 研究继发性淀粉样变的临床特点、治疗与预后。方法 对诊为继发性淀粉样变的全部住院病例进行回顾性临床分析。结果 在 8例继发性淀粉样变病人中 ,其原发病分别为结缔组织病 4例 ,多发性骨髓瘤 3例 ,慢性乙型肝炎 1例。由结缔组织病至继发淀粉样变平均 6 2年 ,肾脏为其主要累及器官 ,表现为蛋白尿 (4例 ) ,1例发生肾功能衰竭 ,以免疫抑制剂及 /或激素治疗后 ,患者平均生存期长于 10年。多发性骨髓瘤继发淀粉样变的时间较短 ,以舌组织及心肌受累为主 ,化疗效果差 ,病人的生存期平均不超过 2年。而继发于慢性肝炎后的淀粉样变主要表现为胸腔积液与肝脾大 ,病情相对平缓。结论 淀粉样变可以继发于结缔组织病、多发性骨髓瘤或慢性炎性疾病 ,他们的起病时间、受累脏器以及临床表现差异很大 ,继发于多发性骨髓瘤的淀粉样变预后差 。

Objective To study the clinical features,treatment and prognosis of secondary amyloidosis.Methods All the inpatients diagnosed as secondary amyloidosis were analyzed retrospectively.Results Eight patients were diagnosed as secondary amyloidosis.Their underlying diseases were connective tissue diseases (4 cases) multiple myeloma (3 cases) and chronic hepatitis B (1 case).Average duration from the diagnosis of connective tissue diseases to secondary amyloidosis was 6 2 years.Kidney was the main involved organ with the manifestation of proteinuria (4 cases) and renal failure occurred in 1 case.Their average life span was longer than 10 years after immunosuppressive agents and/or steroid therapy.Secondary amyloidosis occurred after multiple myeloma showed a bad prognosis.Tongue and myocardium were mainly involved.Enlargement of liver and spleen is the prominent manifestation in the patients with amyloidosis secondary to chronic hepatitis.Conclusion Amyloidosis can be evolved from connective tissue diseases,multiple myeloma or chronic inflammatory diseases.There are differences in clinical manifestations and prognosis.The only treatment of secondary amyloidosis is the early management to its underlying disease.