Acute upper gastrointestinal bleeding due to portal hypertension in a patient with primary myelofibrosis: A case report

Abstract

Background: Acute upper gastrointestinal bleeding is a common medical emergency that has a 10% hospital mortality rate. According to the etiology, this disease can be divided into acute varicose veins and nonvaricose veins. Bleeding from esophageal varices is a life-threatening complication of portal hypertension. Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg. Cirrhosis is the most common cause of portal hypertension, and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western world. Primary myeloproliferative disorders are the main cause of portal venous thrombosis, and somatic mutations in the Janus kinase 2 gene (JAK2 V617F) can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% of primary myelofibrosis.

Case summary: We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis. Peripheral blood tests revealed the presence of the JAK2 V617F mutation. Bone marrow biopsy ultimately confirmed the diagnosis of myelofibrosis (MF-2 grade).

Conclusion: In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis, the possibility of myeloproliferative neoplasms should be considered, and the JAK2 mutation test should be performed.

Keywords: Acute esophageal variceal bleeding; Case report; JAK2 V617F mutation; Myelofibrosis; Portal hypertension.

Figure 1

Examinations images. A and B: Contrast-enhanced abdominal computerized tomography image demonstrating extensive splanchnic vein thrombosis involving the portal, splenic, and superior mesenteric veins; C amd D: Bone marrow biopsy revealed that myelofibrosis (MF-2 grade) was accompanied by a significant increase in platelet count; E and F: An emergency endoscopy showed esophagogastric varices (F3CbRc+Les, severe). A white thrombus was detected on the middle of the esophageal varices.