Background/Aims: Bleeding esophageal varices are a common complication of portal hypertension in patients with underlying liver disease. Often patients with hepatic cirrhosis have hypersplenism with thrombocytopenia and leukopenia. Felty's syndrome is a disorder where patients with rheumatoid arthritis develop splenomegaly, neutropenia, and on rare occasions, portal hypertension without underlying cirrhosis. Methods: We present a case of a patient with portal hypertension secondary to Felty's syndrome and discuss the importance of recognizing this condition since the treatment of choice is surgical management with splenectomy. A review of the literature and underlying liver histologic features are discussed. Results: Medical and surgical management of patients with Felty's syndrome is different from those with portal hypertension due to cirrhosis. Conclusion: Splenectomy is the treatment of choice for complications of portal hypertension in patients with Felty's Syndrome.
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