title: Pulmonary veno-occlusive disease in pulmonary Langerhans' cell granulomatosis.
abstract: This report describes unusual clinical and pathological findings in a 29-yr-old female with pulmonary Langerhans' cell granulomatosis (LCG). During a 7-yr clinical course her condition deteriorated despite corticosteroid therapy, and she died of respiratory failure and pulmonary hypertension. At autopsy, there were widespread pulmonary veno-occlusive disease (PVOD) lesions as well as abundant advanced and healed lesions of pulmonary LCG composed of multiple cysts and stellate fibrosis. The present case demonstrates that pulmonary Langerhans' cell granulomatosis should be considered as a possible cause of pulmonary veno-occlusive disease.
authors.affiliation: Dept of Environmental Health, Harvard School of Public Health, Boston, MA, USA.
authors.forename: K
authors.initials: K
authors.lastname: Hamada
authors.forename: S
authors.initials: S
authors.lastname: Teramoto
authors.forename: N
authors.initials: N
authors.lastname: Narita
authors.forename: E
authors.initials: E
authors.lastname: Yamada
authors.forename: K
authors.initials: K
authors.lastname: Teramoto
authors.forename: L
authors.initials: L
authors.lastname: Kobzik
mesh_terms: D000328:Adult; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D011668:Pulmonary Veno-Occlusive Disease / Q000209:etiology* / Q000473:pathology; D013997:Time Factors