The Hepatologist in the Haematologist Field: a Rare Cause of Non-Cirrhotic Portal Hypertension

An 81 year-old gentleman was admitted to the emergency department of our hospital due to hematemesis and melena, and endoscopic evidence of portal hypertension. Due to presence of ascites and esophageal varices he was referred to gastroenterology care. His history included systemic mastocytosis, and biochemistry showed marked cholestasis with normal liver function tests. Clinical work-up was not consistent with liver disease-related portal hypertension, but rather hepatic involvement of systemic mastocytosis and the development of non-cirrhotic portal hypertension. This clinical case highlights that although systemic mastocytosis is uncommon, the subsequent development of liver involvement and non-cirrhotic portal hypertension are complications of the disease that need always be considered