Gastric varices due to sinistral portal hypertension in a patient with myelofibrosis

Abstract

Introduction: Left-sided portal hypertension (LSPH) is an uncommon cause of gastric varices, most often related to pancreatic disease. However, in patients with myeloproliferative disorders, it may present with isolated gastric varices and upper gastrointestinal bleeding, representing a diagnostic and therapeutic challenge.

Case Presentation: We report the case of a 50-year-old man with myelofibrosis and chronic splenic vein thrombosis who presented with melena and severe anemia. Upper endoscopy revealed isolated gastric varices (GOV2) without esophageal involvement. Doppler ultrasound showed splenomegaly and preserved liver morphology. Contrast-enhanced CT demonstrated chronic splenic vein thrombosis with collateral splenogastric circulation. Hepatic venous pressure gradient was normal, and liver biopsy ruled out cirrhosis. Initial management included transfusion support and terlipressin. A multidisciplinary team recommended partial splenic artery embolization as a less invasive alternative to splenectomy, given the risk of extramedullary hematopoiesis in liver and lung associated with the underlying hematologic disease. Despite this approach, the patient developed early rebleeding, requiring definitive splenectomy, with favorable postoperative recovery.

Discussion: This case highlights the importance of considering non-pancreatic etiologies in the differential diagnosis of isolated gastric varices. In this patient, myelofibrosis-associated hypercoagulability and chronic splenic vein thrombosis were the underlying mechanisms of LSPH. Therapeutic management was tailored to comorbidities, underscoring the role of a stepwise, multidisciplinary approach.

Conclusion: LSPH secondary to myelofibrosis is a rare and challenging entity. Individualized management strategies, adapted to the patient’s underlying pathophysiology, are essential to optimize outcomes in non-cirrhotic portal hypertension.