Masur Y, Remberger K, Hoefer M
Institute of Pathology, University of the Saarland, Homburg/Saar, F.R.G.
Pathol Res Pract. 1996 Mar;192(3):290-5; discussion 296-9. doi: 10.1016/S0344-0338(96)80232-9.
Pulmonary capillary hemangiomatosis (PCH), first described by Wagenvoort et al. in 1978, is a rare cause of pulmonary hypertension which occurs predominantly in young adults. In the literature only 18 cases have been reported. In 1988 Langleben et al. proposed a hereditary form of PCH with probable autosomal-recessive transmission. Histologic findings include irregular small nodular foci of thin-walled capillary-sized vessels which diffusely invade the lung parenchyma, the bronchial/bronchiolar walls and the adventitia of large vessels. We report on a case of PCH in a 24 year old man who presented the clinical signs of interstitial lung disease.
肺毛细血管血管瘤病（PCH）于1978年由瓦根沃特等人首次描述，是导致肺动脉高压的一种罕见病因，主要发生于年轻人。文献中仅报道了18例。1988年，兰勒本等人提出了一种可能为常染色体隐性遗传的遗传性PCH形式。组织学检查结果包括薄壁毛细血管大小的血管形成不规则小结节病灶，这些病灶弥漫性侵犯肺实质、支气管/细支气管壁及大血管外膜。我们报告一例24岁男性的PCH病例，该患者表现出间质性肺疾病的临床症状。
