Idiopathic portal hypertension with transfusion-associated hemolytic anemia: a case report

Idiopathic portal hypertension (IPH) is a rare non-cirrhotic cause of non-cirrhotic portal hypertension, characterized by splenomegaly, hypersplenism, and recurrent variceal bleeding. Transfusion-associated hemolytic anemia with autoimmune features following red blood cell transfusion is an uncommon but serious complication, and its occurrence in IPH patients undergoing invasive procedures has rarely been reported. This study aims to report a rare case of idiopathic portal hypertension complicated by transfusion-associated hemolytic anemia and to describe the clinical outcomes of covered stent transjugular intrahepatic portosystemic shunt followed by glucocorticoid therapy.

A 20-year-old woman with a 3-year history of recurrent hematemesis and melena presented with acute upper gastrointestinal bleeding. She had chronic anemia (hemoglobin 56 g/L), severe splenomegaly crossing the anterior midline, and hypersplenism (white blood cell 1.53×10⁹/L, platelets 35×10⁹/L). Liver function and coagulation were preserved, and contrast-enhanced computed tomography (CT) showed portal hypertension without cirrhosis. Comprehensive workup excluded viral hepatitis, autoimmune liver disease, Budd-Chiari syndrome, and myeloproliferative neoplasms, leading to a diagnosis of IPH. She underwent covered stent transjugular intrahepatic portosystemic shunt (TIPS) placement, with portal pressure decreasing from 41 to 30 cmH₂O. On postoperative day one, after transfusion of washed red blood cells, she developed acute jaundice, dark urine, and worsening anemia (hemoglobin 46 g/L). Laboratory findings revealed indirect hyperbilirubinemia (indirect bilirubin 72.0 µmol/L) and positive direct/indirect anti-human globulin tests, confirming transfusion-associated hemolytic anemia with autoimmune features. She was treated with methylprednisolone (40 mg/day) followed by oral prednisone tapered over 3 months, along with urinary alkalinization and choleretic agents. Hemolysis resolved within 3 days, and no recurrent bleeding occurred despite recent variceal hemorrhage. At 12-month follow-up, the TIPS remained patent, gastroscopy confirmed absence of esophageal varices, and hypersplenism parameters normalized without need for splenectomy.

Covered stent TIPS may effectively control portal hypertension and improve hypersplenism in select IPH patients. However, transfusion-associated hemolytic anemia is a potential complication in alloimmunized patients receiving transfusions during TIPS, requiring prompt diagnosis and glucocorticoid therapy. Multidisciplinary collaboration among hepatologists, hematologists, and interventional radiologists is essential for optimal outcomes.