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目的 提高临床和病理医生对特发性肺含铁血黄素沉着症的认识。方法 回顾分析9例特发性肺 含铁血黄素沉着症的临床表现、误诊情况、治疗与转归。结果 9例患者中4例强的松治疗3～7周后缓解,1例强 的松治疗2月无效后加用环磷酰胺治疗缓解,4例治疗无效死亡。结论 特发性肺含铁血黄素沉着症是一种少见 病,发病原因迄今无确切认识。目前尚无特异疗法,病死率高,儿童预后较成人差。
Objective To improve the understanding of clinicians and pathologists on idiopathic pulmonary hemosiderosis. Methods Retrospective analysis of 9 cases of idiopathic pulmonary hemosiderosis clinical manifestations, misdiagnosis, treatment and prognosis. Results Of the 9 patients, prednisone was relieved in 4 of 3 patients after treatment for 3 to 7 weeks. One case of prednisone was ineffective in 2 months and was treated with cyclophosphamide. Four patients died of treatment ineffectively. Conclusions Idiopathic pulmonary hemosiderosis is a rare disease, so far the exact cause of the disease is unknown. There is no specific therapy, high mortality, poor prognosis in children than adults.