POEMS syndrome presenting as Porto-sinusoidal vascular disease: A case report

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.