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特发性肺含铁血黄素沉着症与肺泡出血综合征:病例报告及文献综述

Idiopathic pulmonary hemosiderosis and alveolar hemorrhage syndrome: case report and review of the literature.

作者信息

Rezkalla M A, Simmons J L

机构信息

VA Hospital, Sioux Falls, SD, USA.

出版信息

S D J Med. 1995 Mar;48(3):79-85.

PMID:7740300
Abstract

The presence of widespread hemorrhage from the microvasculature of the lung into the alveolar spaces defines what is called the "alveolar hemorrhage syndrome", which can occur in association with a wide variety of clinical disorders. The cardinal manifestations of this syndrome include: hemoptysis, unexplained anemia and diffuse alveolar infiltrates on chest roentgenograms. Since the pulmonary features are similar, the diagnosis usually depends on the clinical, laboratory and pathologic evaluations. Early diagnosis and treatment is crucial since the occurrence of pulmonary hemorrhage in this syndrome may represent a catastrophic event with fatal consequences. Idiopathic pulmonary hemosiderosis (IPH) has been identified as a cause of alveolar hemorrhage in a small number of cases, mainly by exclusion criteria. We report a case of a 70-year old man who presented with a 40-year history of intermittent hemoptysis and bilateral upper lobes alveolar infiltrates proved to be secondary to idiopathic pulmonary hemosiderosis. Although the lung apices are frequently spared in IPH, they were the site of the infiltrates in our case. To our knowledge, our patient had the longest survival time ever reported in the literature in adult IPH. A brief review of some of the disorders commonly associated with alveolar hemorrhage is also presented.

摘要

肺微血管广泛出血进入肺泡腔的情况定义为所谓的“肺泡出血综合征”,它可与多种临床疾病相关。该综合征的主要表现包括:咯血、不明原因的贫血以及胸部X线片上的弥漫性肺泡浸润。由于肺部特征相似,诊断通常取决于临床、实验室和病理评估。早期诊断和治疗至关重要,因为该综合征中肺出血的发生可能是一个灾难性事件,会导致致命后果。特发性肺含铁血黄素沉着症(IPH)在少数病例中被确定为肺泡出血的一个原因,主要是通过排除标准。我们报告一例70岁男性患者,有40年间歇性咯血病史,双侧上叶肺泡浸润经证实继发于特发性肺含铁血黄素沉着症。尽管IPH中肺尖部通常不受累,但在我们的病例中浸润部位却是肺尖部。据我们所知,我们的患者是文献报道中成年IPH患者存活时间最长的。本文还简要回顾了一些通常与肺泡出血相关的疾病。

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