临床诊断肺毛细血管瘤病一例报道并文献复习

摘要

肺毛细血管瘤病(PCH)是一种罕见的肺组织内血管增生性疾病,主要病理表现为大量薄壁毛细血管增生并浸润肺泡壁、气道、血管甚至胸膜,偶可侵及纵隔及心包,且增生的毛细血管常沿肺泡壁排列并包绕甚至压迫肺血管,可导致肺小静脉内膜增厚和肺小动脉血管重塑等,患者预后多不良.本文报道了1例PCH患者并复习相关文献以分析其可能的发病机制及诊治要点,以提高临床对该病的认识,减少误诊、漏诊.%Objective Pulmonary capillary hemangiomatosis is one of pulmonary vascular proliferative diseases,it is rare and its prognosis is mostly poor.Main pathological manifestation of pulmonary capillary hemangiomatosis includes plenty of thin-walled capillaries hyperplasia with infiltrations of alveolar wall,airway,blood vessel and pleura,some patients may complicate with infiltrations of mediastinum and pericardium;the hyperplastic capillaries usually lined up along the alveolar wall with circumvolution or oppression on pulmonary vessels,which may result in pulmonary venules intima thickening and pulmonary arterioles remodeling.This paper reported a patient clinically diagnosed as pulmonary capillary hemangiomatosis and reviewed related literatures to analyze the possible pathogenesis,main points of diagnosis and treatment,thus to improve the clinical acquaintance,reduce misdiagnosis and missed diagnosis.