弥漫性肺泡出血的临床和病理学特征  

Ciinicopathalogical diagnosis of diffuse alveolar hemorrhage

作　　者：方芳[1] 李燕明[2] 胡松涛[1] 王海涛[3] 刘东戈[1] 王辰[2] 

机构地区：[1]北京医院病理科,100730 [2]北京医院呼吸与危重症医学科,100730 [3]北京医院肾内科,100730

出　　处：《中华医学杂志》2016年第2期108-112,共5页National Medical Journal of China

基　　金：国家科技支撑计划（2012BAI05B02）

摘　　要：目的探讨弥漫性肺泡出血（DAH）的临床和病理学特征。方法回顾性分析1999年5月至2015年5月北京医院有完整病理资料的6例DAH患者的临床资料。其中男2例，女4例；年龄32～68岁，平均58．8岁。尸检3例、开胸肺活检2例和肾穿刺活检2例（其中1例于2003年开胸肺活检、于2012年肾穿刺活检）。结果临床主要表现为咳嗽、气促，呼吸困难。其中咯血5例，高热4例，皮肤黏膜出血3例，肉眼血尿及镜下血尿各2例；血肌酐升高3例，红细胞沉降率和c反应蛋白不同程度升高5例。6例均有中度贫血、低氧血症和影像学双肺弥漫性病变。血清学抗中性粒胞质抗体阳性（ANCA）3例，抗肾小球基底膜抗体阳性1例。病理学结果：肉芽肿性血管炎2例，显微镜下多血管炎2例，Goodpasture综合征1例，肺静脉闭塞性疾病1例。预后：死亡3例；好转出院2例；对症治疗、出院观察1例。结论DAH主要表现为不同程度的呼吸困难、贫血、低氧血症和影像学双肺弥漫性病变，伴或不伴咯血，肺组织弥漫性新鲜或陈旧性出血为其主要病理学特征。Objective To improve knowledge about the clinical and pathological features of diffuse alveolar hemorrhage （DAH）. Methods Six cases DAH with intact clinical and pathological data were retrospectively analyzed during the period from May 1999 to May 2015 in Beijing Hospital. There were altogether 2 males and 4 females, with age ranging from 32 to 68 years （ mean 58. 8 years）. Specimens were obtained by autopsy （3 cases）, open lung biopsy （2 cases） and renal biopsy （2 cases）, including 1 case of open lung biopsy in 2003, renal biopsy in 2012. Results Clinically, the patients presented with cough, shortness of breath and dyspnea, including 5 cases of hemoptysis, 4 cases of fever, 3 cases of skin and mucosa bleeding, 2 cases of gross hematuria, 2 cases of microscopic hematuria, 3 cases of renal functional impairment. A total of 5 cases had different levels of elevated erythrocyte sedimentation rate and C-reactive protein, 6 cases had moderate anemia, hypoxemia, diffuse infiltrates with alveolar filling in chest CT. Serum antineutrophil cytoplasmic antibody was positive in 3 cases, anti-glomerular basement membrane antibody was present in 1 case. Pathological diagnosis ： 2 cases of Granulomatosis with polyangiitis （ GPA）, 2 cases of Microscopic polyangiitis （ MPA）, 1 case of Goodpasture syndrome, 1 case of pulmonary veno-occlusive disease （PVOD）. Prognosis： 3 cases died; 2 cases were discharged; 1 case received symptomatic treatment, follow-up after discharge. Conclusion The mainly clinical characteristics of DAH are varied degree of dyspnea, anemia, hypoxemia, and extensive ground-glass opacification or consolidation in image, with or without haemoptysis; diffuse acute or chronic pulmonary hemorrhage in lung tissue is the main pathological feature.

关 键 词：肺泡 出血 疾病特征 病理学 

分 类 号：R563[医药卫生—呼吸系统]