Plexogenic pulmonary hypertension associated with POEMS syndrome. (2017)

• Record Type: Journal Article
• Authors: Czeczok, Thomas; Lin, Peter; Yi, Eunhee
• Is Part Of: Respiratory medicine case reports. Volume 22(2017)
• Page Start: 168
• Page End: 170
• Publication Date: 2017
• Subjects: Plexiform -- Pulmonary hypertension -- POEMS; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2017.08.011
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: Pulmonary hypertension is one of the well-known clinical manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, occurring in approximately 25–30% of the affected individuals. However, the histopathologic spectrum of pulmonary hypertension associated with POEMS syndrome has not been fully documented in the literature. Herein, we report an autopsy case of POEMS syndrome in a patient whose lung tissues showed histopathology indistinguishable from that of idiopathic pulmonary arterial hypertension with abundant plexiform lesions in the small pulmonary arteries.