淀粉样变性71例临床分析

A clinical analysis of 71 cases of amyloidosis

目的 提高临床对淀粉样变性 (AD)的认识。方法 回顾性分析了 1982年 2月至 2 0 0 2年 2月经组织活检和临床证实符合AD诊断的患者 71例。结果 系统性AD 5 7例 ,5 7例中原发性AD 33例 ,继发性AD 2 2例 ,家族性AD多神经病 2例 ;局限性AD 14例。 82 %的系统性AD表现为心脏彩超异常 ,腹壁脂肪及齿龈活检刚果红染色阳性率分别为 80 0 %和 87 5 %。系统性AD多用马法兰和泼尼松 (MP)或MP +秋水仙碱治疗 ,局限性患者采用观察或局部手术切除。 15例系统性AD住院期间死亡 ,其中 5例死于心力衰竭。结论 系统性AD累及多系统器官 ,临床表现多样 ,预后较差 ,而局限性预后较好。因此区分AD为系统性抑或局限性非常重要。齿龈或腹壁脂肪活检是安全而有效的活检部位 ,心脏彩超对诊断也很有帮助。

Objective To comprehend the clinical characteristics and treatment of amyloidosis.Methods The clinical data of 71 patients with amyloidosis ,admitted to Peking Union Medical College Hospital from Feburary 1982 to Feburary 2002 ,were analyzed. Results 57 of the 71 cases were systemic amyloidosis. Among the 57 cases, 33 were primary systemic amyloidosis , 22 secondary systemic amyloidosis and 2 familial amyloid polyneuropathy.The remaining 14 cases were localized amyloidosis.82% of the patients with systemic amyloidosis showed adnormalities in Doppler echocardiography. The positive rate of biopsy of subcutaneous fat and gingiva was 80.0% and 87.5% respectively. The treatment of systemic amyloidosis was chemotherapy,and that of localized amyloidosis was observation or localized excision .15 systemic amyloidosis patients died during hospitalization, mainly due to congestive heart failure. Conclusion As treatment and prognosis are different between local and systemic amyloidosis ,the distinction between them is very important. Biopsy of abdominal fat and gingival is a useful and safe procedure to identify patients with systemic disease. Doppler echocaridiography examinition is also helpful for the diagnosis.