Portal hypertension caused by essential thrombocythemia: A case report

Portal hypertension (PHT) results from increased intrahepatic vascular resistance and augmented portal venous blood flow. Although cirrhosis remains the predominant etiology of PHT, non-cirrhotic PHT also requires clinical attention. This article reports a rare case of PHT with portal vein thrombosis and gastroesophageal varices secondary to essential thrombocythemia (ET).

An 82-year-old female patient was admitted due to "hematemesis and melena," abdominal computed tomography revealed ascites, altered liver morphology, mild splenomegaly and PHT. The patient was admitted with a diagnosis of "decompensated cirrhosis." She maintained normal liver function over a 6-month follow-up, subsequently, the condition progressed to portal vein thrombosis, leading to gastrointestinal bleeding. Laboratory assessments at this stage revealed normal levels of albumin, liver enzymes, bilirubin, and coagulation factors, with no ascites formation, inconsistent with decompensated cirrhosis.

ET was diagnosed based on Bone Marrow Aspiration and Biopsy, the JAK2 mutation was identified with a variant allele frequency of 42.2%. PHT and thrombosis in this case were attributed to ET.

The patient was treated with hydroxyurea and ruxolitinib, endoscopic injection cyanoacrylate/sclerotherapy and endoscopic variceal ligation were performed.

Post-discharge, oral antitumor agents and aspirin for antiplatelet therapy were prescribed, with outpatient follow-up.

ET can also lead to PHT, portal vein thrombosis, and esophageal varices. Clinicians should consider these possibilities when dealing with such patients.