成人特发性肺含铁血黄素沉着症5例临床分析

摘要

Objective To study the rliniral features oi idiopathir pulmonary hemosiderosis ( IPH ) in adults and to evaluate the methods of diagnosis and treatment. Methods Clinical data about 5 rases of IPH( 3 males and 2 females ) were retrospectively analyzed with its related literature reviewed. Results The onset age oi IPH was 29. 2 years( mean 22 ～ 39 years ). All oi these patients had rhest disties, the main eliniral manifestations included cough and hemosputum in 2 cases, hemoptysis in 2 cases, and fever in 2 case. Chest radiogram showed bilateral diiiuse alveolar opacities over mid and lower zones. Numerous hemosiderin laden macrophages in the sputum or broncho alveolar lavage fluid( BALF )were seen. Conclusions There is no specific clinical maniiestations in adults with IPH. Chest radiogram and finding the hemosiderin cells in. sputum or BALF are helpiul in diagnosis.%目的 探讨成人特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH )的临床特征、诊断和治疗方法.方法 分析5例IPH患者的临床资料并复习文献.结果 5例患者中,男3例,女2例;发病年龄22~39岁,平均年龄29.2岁;5例患者均表现有胸闷、气短,主要临床表现包括咳嗽、咳痰、痰中带血3例,反复咯血2例,发热2例;胸部影像4例呈毛玻璃状改变,其中3例散在分布粟粒样结节,1例仅表现为双肺腺泡样结节影;5例均在痰或者肺组织中查到含铁血黄素巨噬细胞.结论 IPH临床表现缺乏特异性,影像学和反复痰或肺泡灌洗液中含铁血黄素细胞检查有助于诊断.