Pulmonary fibrosis in Hermansky-Pudlak syndrome is not fully usual
Pulmonary fibrosis in Hermansky-Pudlak syndrome is not fully usual
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DOI：
10.1016/s0242-6498(06)70753-2
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发表时间：
2006-12-01
影响因子：
0.5
通讯作者：
Dartevelle, Philippe
中科院分区：
文献类型：
--
作者：
de Montpreville, Vincent Thomas;Mussot, Sacha;Dartevelle, Philippe
一名 55 岁女性 Hermansky-Pudlak 综合征（白化病、血栓病和蜡样积聚）患者因肺纤维化接受了单次肺移植。对移植肺的检查显示出常见的间质性肺炎模式，并伴有两种不寻常的病变：纤维化中存在大量含有蜡质色素的巨噬细胞，以及肺细胞的特征性泡沫状肿胀。这种后来的病变最近才被描述，似乎与表面活性剂分泌缺陷的疾病机制有关。这种病变允许与特发性普通间质性肺炎的赫曼斯基-普德洛克综合征相关的肺纤维化的组织学个体化。
A 55-year-old female patient with Hermansky-Pudlak syndrome (albinism, thrombopathia and ceroid accumulation) underwent a single lung transplantation for pulmonary fibrosis. Examination of explanted lung showed usual interstitial pneumonia pattern associated with two unusual lesions: presence Of numerous macrophages containing ceroid pigments within fibrosis and characteristic foamy swelling of pneumocytes. This later lesion, which has only been recently described, seems related to the mechanism of the disease by defect of surfactant secretion. This lesion allows the histological individualization of pulmonary fibrosis associated with Hermansky-Pudlok syndrome from idiopathic usual interstitial pneumonia.