pulmonary capillary hemangiomatosis
Capillary proliferations in the pulmonary alveolar walls. Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension.
Definition: Pulmonary capillary hemangiomatosis (PCH) is characterized by proliferating capillaries that invade the pulmonary interstitium and alveolar septae, and occlude the pulmonary vasculature. It is a rare idiopathic lung disorder that occurs in young patients and leads to pulmonary hypertension (PH).
Synopsis
proliferating capillaries invading the pulmonary interstitium and alveolar septae, and occluding the pulmonary vasculature.
capillary proliferations in the pulmonary alveolar walls
- Capillary proliferation is particularly well demonstrated by CD34
numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through alveolar walls and in and around larger vessels and airways
venous infiltration with intimal fibrosis and secondary veno-occlusive disease
proliferation of benign thin-walled capillary sized blood vessels in the lung parenchyma
atypical endotheliomatosis
mild interstitial lymphocytic infiltrate
no venulitis or infarction
Associations
hereditary hemorrhagic telangiectasia (HTT) (#14991548#)
Variants
congenital pulmonary capillary hemangiomatosis (#12910588#)
diffuse bilateral pulmonary capillary hemangiomatosis after bilateral lung transplantation (#12821168#)
See also
pulmonary veno-occlusive disease
References
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Am J Surg Pathol. 2006 Jul;30(7):850-7. PMID: #16819327#
Tron V, Magee F, Wright JL, Colby T, Churg A. Pulmonary capillary hemangiomatosis. Hum Pathol. 1986 Nov;17(11):1144-50. PMID: #3770733#