特发性肺含铁血黄素沉着症13例临床分析

目的 探讨特发性肺含铁血黄素沉着症的I临床表现,以提高对本病的认识.方法 回顾性分析2007年12月以前北京协和医院出院诊断为特发性肺含铁血黄素沉着症的13例临床资料.结果 13例患者中,儿童及青少年8例.成人5例;男性9例,女性4例.临床表现方面,8例出现不同程度的反复咳血.11例胸部CT检查最主要的征象为双肺弥漫病变或间质改变(9/11).其次为磨玻璃样改变(5/11),双肺弥漫或双下肺为主,也可呈中心性蝶翼状或有游走征象.再次为小结节状或粟粒状致密阴影(5/11),其他征象有细网格状影(3/11),絮状影(1/11)等.5例支气管肺泡灌洗液(bronchoalveolar lavage fluid,BALF)结果.其中BALF呈暗红色、灰褐色或血性(5/5),细胞学检查可见大量红细胞(3/5),吞噬含铁血黄素细胞(5/5).8例病理结果主要表现为肺泡腔内可见大量吞噬含铁血黄素的巨噬细胞(7/8),肺泡Ⅱ型上皮细胞增生(5/8),肺间质纤维组织增生.慢性炎症细胞聚集(5/8),肺泡间隔增厚(2/8)等.结论 特发性肺含铁血黄素沉着症常于儿童及青少年时期起病,男性多于女性.最突出的临床表现为咳血,胸部CT特征为双肺弥漫病变,磨玻璃样改变和结节状或粟粒状致密阴影.依靠肺组织活检病理进行排他性诊断是诊断该病的关键.

Clinical study of 13 cases of idiopathic pulmonary haemosiderosis

Objective To study the clinical features of diopathic pulmonary haemosiderosis. Methods Restrospective review was made on 13 cases with the diagnosis of idiopathic pulmonary haemosiderosis (IPH) in ward. The clinic data of the 13 patients including clinical, physical, laboratory, radiological, pathological and pulmonary function presentations were collected. Results 8 out of 13 cases were childhood-onset(<18 years). Sex distribution appeared to a male predominance. The most important symptom was variable degrees of repetitive haemoptysis (8/13). The signs of CT scan showed from diffused interstitial changes (9/11) to ground-glass opacities (5/11) and micronodular patterns (5/11). The main cellular type of the bronchoalveolar lavage fluid was the alveolar macrophage filled with haemosiderrin (5/5). The pathologic manifestations on light microscopy of lung biopsy were the presence of haemosiderrin-laden macrophages in alveoli (7/8), type two pneumocytes hyperplastic (5/8) and fibroplastic proliferation in interstitium (5/8). Conclusions IPH patients are typically children,often present with haemoptysis. The characteristics of CT scan are diffused interstitial changes,ground-glass opacities and micronodular patterns. The pathologic manifestations are the presence of haemosiderrin-laden macrophages in alveoli, type two pneumocytes hyperplastic and fibroplastic proliferation in interstitium. The exclusion of other diseases is important to the diagnosis.