A rare case of pulmonary hypertension: Pulmonary capillary hemangiomatosis

Abstract

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary arterial hy pertension and a benign vascular neoplasm characterized by thin walled microvessels infiltrating the peribronchial and perivascular interstitium, the lung parenchyma, and the pleura. At compu terised tomography of lung, main pulmonary arterial enlargement and widespread ill defined cen trilobular nodules of ground glass opacity are consistently described, often mixed with lobular ground glass opacities. Pathological diagnosis requires surgical biopsy, but the compromised con dition of the patients makes this hazardous. Majority of the reported cases have been discovered postmortem due to lack of awareness and difficulty in making that diagnosis. Hereby, a young wo man who died with hematologic complications due to PCH was reported. Copyright © 2013 by Türkiye Klinik leri.