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[肺泡蛋白沉积症:4例超微结构研究]

[Pulmonary alveolar proteinosis: ultrastructural study of 4 cases].

作者信息

Garrido L, Gledhill T, Martin M, Caleiras E, Parada D, García Tamayo J

机构信息

Departamento de Patología, Hospital José Ignacio Baldó, Caracas, Venezuela.

出版信息

Invest Clin. 1997 Mar;38(1):25-37.

PMID:9235071
Abstract

Pulmonary Alveolar Proteinosis (PAP) is a rare and diffuse lung disease characterized by the abnormal deposition of PAS positive, lipoproteinaceous material in the alveolar spaces. It has been related, mainly, to alterations in the immune state and to secondary infections. We studied four cases of PAP diagnosed by light microscopy. In two cases we were able to demonstrate disseminated Histoplasmosis related to immunodeficiency states (AIDS and malnutrition), one case with Pneumocystis carinii infection and AIDS, and one case with no related pathology. Granular and electron dense material, concentric myelin figures, and variable-sized osmiophilic bodies were observed by electron microscopy. We found yeast-like structures, trophozoites and cysts in the alveolar spaces, in the Histoplasmosis and Pneumocystic carinii infection cases, respectively. In one of our cases, the circulating neutrophils showed crystalloid inclusions in the nucleus. PAP should be considered in the differential diagnoses of patients with pulmonary infiltrates.

摘要

肺泡蛋白沉积症(PAP)是一种罕见的弥漫性肺部疾病,其特征是肺泡腔内出现过碘酸雪夫(PAS)染色阳性的脂蛋白物质异常沉积。它主要与免疫状态改变及继发感染有关。我们研究了4例经光镜诊断的肺泡蛋白沉积症病例。其中2例证实为与免疫缺陷状态(艾滋病和营养不良)相关的播散性组织胞浆菌病,1例为卡氏肺孢子虫感染合并艾滋病,1例未发现相关病理改变。通过电子显微镜观察到颗粒状和电子致密物质、同心性髓鞘样结构以及大小不等的嗜锇小体。在组织胞浆菌病和卡氏肺孢子虫感染病例中,分别在肺泡腔内发现了酵母样结构、滋养体和包囊。在我们的1例病例中,循环中的中性粒细胞细胞核内可见晶体样包涵体。对于肺部浸润患者的鉴别诊断应考虑肺泡蛋白沉积症。

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