Tron V, Magee F, Wright J L, Colby T, Churg A
Hum Pathol. 1986 Nov;17(11):1144-50. doi: 10.1016/s0046-8177(86)80420-8.
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension; only three cases have been reported. Four additional cases are described in this report. All of the patients in this study presented with signs and symptoms of pulmonary hypertension, and in none was a correct morphologic diagnosis made during life. Histologically, the most striking feature was the presence of numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through alveolar walls and in and around larger vessels and airways. Venous infiltration was associated with intimal fibrosis and secondary veno-occlusive disease (VOD). Because of the presence of hemorrhage, the apparent interstitial widening and inflammation, and the venous changes, the condition in these cases was initially misdiagnosed as interstitial fibrosis or VOD. However, the identification of proliferating and invasive capillaries, which are unique to PCH, led to the correct diagnosis. Although the nature of PCH is unknown, it behaves like a low-grade vascular neoplasm.
肺毛细血管血管瘤病（PCH）是肺动脉高压的一种罕见病因；仅报告过3例。本报告描述了另外4例。本研究中的所有患者均表现出肺动脉高压的体征和症状，且生前均未做出正确的形态学诊断。组织学上，最显著的特征是存在大量细胞学上良性的薄壁毛细血管大小的血管，这些血管弥漫性地通过肺泡壁以及在较大血管和气道内及周围增殖。静脉浸润与内膜纤维化和继发性静脉闭塞性疾病（VOD）相关。由于存在出血、明显的间质增宽和炎症以及静脉改变，这些病例最初被误诊为间质纤维化或VOD。然而，PCH特有的增殖性和侵袭性毛细血管的识别导致了正确的诊断。尽管PCH的性质尚不清楚，但它表现得像一种低度血管肿瘤。
