POEMS syndrome presenting as Porto-sinusoidal vascular disease: A case report - 16/04/26
Abstract |
POEMS syndrome presenting initially as portal hypertension is rare. We report a 49-year-old man who was initially diagnosed with cryptogenic cirrhosis and refractory ascites. Liver biopsy later revealed Porto-sinusoidal Vascular Disease (PSVD), rather than cirrhosis. During the follow-up, he developed polyneuropathy, organomegaly, endocrinopathy, and skin changes. Further evaluation confirmed POEMS syndrome with elevated vascular endothelial growth factor (VEGF) and IgA monoclonal gammopathy. Treatment with prednisone and lenalidomide significantly improved his ascites, eliminating the need for TIPS. This case highlights that POEMS syndrome should be considered in cases of unexplained non - cirrhotic portal hypertension.
Le texte complet de cet article est disponible en PDF.Keywords : POEMS syndrome, Portal hypertension, Porto-sinusoidal Vascular Disease, PSVD
Abbreviations : HVPG, PH, PSVD, SAAG, TIPS, TNF-α, VEGF
Plan
Vol 50 - N° 5
Article 102826- mai 2026 Retour au numéro
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