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伴有肺含铁血黄素沉着症和持续性网织红细胞增多的血管肉瘤。类固醇反应性提示存在免疫基础。

Angiosarcoma with pulmonary siderosis and persistent reticulocytosis. Steroid responsiveness suggests an immune basis.

作者信息

Rajdev N, Green R, Crosby W H

出版信息

Arch Intern Med. 1978 Oct;138(10):1549-51.

PMID:568458
Abstract

A 35-year old man with cough, hemoptysis, and dyspnea was found to have diffuse pulmonary infiltrates and iron-laden macrophages in the sputum. Pulmonary siderosis was confirmed by transbronchial biopsy. An associated hypochromic anemia required frequent transfusion. Though marrow iron stores were absent, reticulocytosis was maintained. Corticosteroid therapy resulted in cessation of hemoptysis, clearing of the pulmonary infiltration, and a substantial reduction in transfusion requirement. Splenectomy was of no benefit. The patient developed cerebral symptoms with seizures, and rapid deterioration led to cerebral symptoms with seizures, and rapid deterioration led to death. Disseminated hemangiosarcoma was found at autopsy. Steroid responsiveness of the associated pulmonary siderosis suggests that it had an immune basis.

摘要

一名35岁男性,有咳嗽、咯血和呼吸困难症状,痰中发现弥漫性肺浸润和含铁血黄素巨噬细胞。经支气管活检确诊为肺铁沉着症。伴有低色素性贫血,需要频繁输血。尽管骨髓铁储存缺乏,但仍维持网织红细胞增多。皮质类固醇治疗使咯血停止,肺部浸润消退,输血需求大幅减少。脾切除术无益处。患者出现癫痫发作的脑部症状,病情迅速恶化,最终导致癫痫发作的脑部症状,病情迅速恶化并导致死亡。尸检发现弥漫性血管肉瘤。相关肺铁沉着症对类固醇有反应,提示其有免疫基础。

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