Thrombophilia and Polycythemia in a Woman With Budd-Chiari Syndrome

A 31-year-old woman with Budd-Chiari syndrome, a condition blocking liver veins, was diagnosed with non-criteria antiphospholipid syndrome, a rare autoimmune disorder causing blood clots, after initial suspicion of a type of blood cancer was ruled out. She showed symptoms like persistent hyperhemoglobinemia, increased hematocrit, and thrombocytopenia. Her condition improved following a transjugular intrahepatic portal shunt procedure and a combination of intravenous methylprednisolone, low–molecular-weight heparin, and warfarin sequential anticoagulant therapy. She is now in sustained remission and awaiting a liver transplant. This case emphasizes the need to consider non-criteria antiphospholipid syndrome in patients with unexplained Budd-Chiari syndrome, particularly those with multiple thromboemboli and thrombocytopenia.