Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocytemia: Case Report

摘要: Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the veins, inferior vena cava, or right atrium. Reports from literature have delineated on focal nodular hyperplasia (FNH)-like lesions association with Syndrome. To our knowledge, there are no reports about true FNH patients Focal develops disorders aberrant circulation and vasculature. We report case of large solitary 22-year-old man who was referred to institution sudden intermittent upper quadrant abdominal pain, vomiting, diarrhea pale stool, decreased appetite, dark urine, distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, abnormal liver function tests coagulation profile. Imaging revealed vein thrombosis, confirming syndrome, 6.2 × 6.1 × 6.8 cm lesion segment 8 liver. Primary cause essential thrombocythemia according bone marrow biopsy and molecular testing results. The patient treated medically underwent transjugular intrahepatic portosystemic shunt insertion. continued enlarge. Cadaveric transplantation carried out. On gross histologic examination explanted liver, found be FNH.