原发性支气管肺淀粉样变4例并文献复习  

Clinical analysis of primary bronchial and pulmonary amyloidosis

作　　者：王平[1] 梁志欣[1] 方向群[1] 徐淑凤[1] 孙继萍[1] 陈良安[1] 

机构地区：[1]中国人民解放军总医院,100853

出　　处：《山西医药杂志（上半月）》2008年第4期313-315,共3页Shanxi Medical Journal

摘　　要：目的探讨原发性支气管肺淀粉样变的临床表现、影像学特征和诊治方法。方法对解放军总医院的4例和1994年以来国内报道的39例原发性支气管肺淀粉样变进行回顾性总结。结果①临床主要表现为:咳嗽(95%,41/43)、咯痰(74%,32/43)、气短(51%,22/43)、咯血(46%,20/43)等。②影像学特征:主要表现为肺部斑片状或结节阴影和气管壁环形增厚、管腔狭窄。③肺功能及实验室检查:肺功能检查以阻塞性通气功能障碍为主,实验室检查无特征性阳性发现。④病理学检查:组织标本见均质嗜伊红物质沉积于支气管和肺,刚果红染色阳性。⑤治疗支气管肺淀粉样变的治疗方法有以马法兰+泼尼松治疗为主的化疗,激光、冷冻和手术治疗以及对症治疗等。结论该病临床表现无明显特征,组织病理检查是确诊的唯一方法;治疗主要有化疗、激光和手术治疗。该病易复发,预后不良。Objective To study the clinical features, treatments, and prognosis of primary bronchial and pulmonary amyloidosis. Methods A retrospective analysis of 43 cases was made, among which four were diagnosed and treated in the Chinese PLA General Hospital and the clinical data of the other thirty-nine cases were studied by literature review. Results Among the 43 patients, 28 were male （65%） and 15 was female （35%）. The age of patients ranged from 28 to 81 years, with an average age of （51 ± 12） years. The main clinical manifestations included cough （41 cases, 95%）, sputum （32 cases, 74%）, short breath （22 cases, 51%） and hemoptysis （20 cases, 46%）. Chest radiography findings of these patients showed multiple nodules, irregular luminal narrowing, and airway wall thickening. Eighteen patients took pulmonary function test with the results of obstructive ventilatory disturbance in 17 cases. The diagnosis of amyloidosis was made using histochemical analysis of bronchial wall or surgery biopsy in all cases. The treatments of the disease included chemotherapy, surgery and laser therapy, and so on. Conclusion Primary bronchial and pulmonary amyloidosis is uncommon, and the abnormalities are considered non-specific.

关 键 词：淀粉样变性 支气管 肺 

分 类 号：R56[医药卫生—呼吸系统]