A unique case of bleeding from esophageal varices as the first sign of essential thrombocythemia

Upper gastrointestinal bleeding (UGIB) is a common cause of hospital admission and variceal hemorrhage is responsible for many UGIB cases. Esophageal and gastric varices are caused by portal hypertension (PHT), mostly due to liver cirrhosis. Portal vein thrombosis (PVT) is an important cause of non-cirrhotic PHT and can be associated with several diseases, including myeloproliferative disorders such as essential thrombocythemia (ET). PVT may become apparent due to complications of PHT, including variceal bleeding (VB). We report the case of a 43-year-old male admitted with esophageal VB. Etiologic work-up for chronic liver disease was negative and abdominal magnetic resonance imaging revealed chronic PVT with cavernous transformation and a non-cirrhotic liver. JAK2 mutation was found, and the bone-marrow biopsy was consistent with ET, without peripheral blood alterations. This is a unique case of ET diagnosed in a variceal bleeding setting, remembering the necessity for high clinical suspicion.