特发性肺含铁血黄素沉着症的诊断与治疗

Diagnosis and Treatment of Idiopathic Pulmonary Hemosiderosis

特发性肺含铁血黄素沉着症是一种罕见的可能致命的弥漫性肺泡内出血性疾病。原因未明,以广泛的肺毛细血管出血为特点,血红蛋白分解后形成的以含铁血黄素形式沉着在肺泡间质,最后导致肺纤维化。多发生于新生儿及儿童,反复咯血、缺铁性贫血和弥散性肺浸润三联征是其特征性表现。经铁染色检测痰、胃液、支气管肺泡灌洗液或肺组织发现含铁血黄素巨噬细胞是最为重要的诊断方法。激素及免疫抑制剂是目前治疗的方法,因其临床表现不具有特异性所以误诊率极高,所以早期诊断十分重要,早期干预可降低病死率。

Idiopathic pulmonary hemosiderosis is a rare,potentially fatal,diffuse alveolar hemorrhagic disease.The reason is unknown.It is characterized by extensive pulmonary capillary hemorrhage.The hemoglobin decomposes and forms hemosiderin in the form of hemosiderin,which eventually leads to pulmonary fibrosis.It occurs mostly in newborns and children.Repeated hemoptysis,iron deficiency anemia and diffuse pulmonary infiltration are characteristic features.The detection of hemosiderin macrophages by iron staining for sputum,gastric juice,bronchoalveolar lavage or lung tissue is the most important diagnostic method.Hormones and immunosuppressive agents are currently the treatment methods.Because their clinical manifestations are not specific,the rate of misdiagnosis is extremely high,so early diagnosis is very important,and early intervention can reduce the mortality rate.