Two DeLIVERies and a transplant: Case report of acute fatty liver of pregnancy requiring liver transplant

Abstract

A 35-year-old G2P1001 female at 35 weeks' gestation with a dichorionic diamniotic twin pregnancy presented with nausea, vomiting, and mild confusion for 1 week. She had a prior uncomplicated pregnancy and no significant past medical history. Her initial bloodwork revealed leukocytosis to 12.9 × 10 3 /μL, thrombocytopenia to 103 × 10 3 /μL, international normalized ratio of 6.8, creatinine of 1.91 mg/dL (baseline 0.5-0.7 mg/dL), lactate dehydrogenase of 1771U/L, hyperbilirubinemia to 8.6 mg/dL, direct bilirubin of 6.7 mg/dL, alanine aminotransferase 327 U/L, aspartate aminotransferase 559 U/L, and alkaline phosphatase 473 U/L. Due to initial concern for hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome, the patient was taken for an emergent Cesarean delivery. The operative course was complicated by postpartum hemorrhage and disseminated intravascular coagulation. Female twin B required a 2-week neonatal intensive care stay for respiratory distress; however, male twin A had an uncomplicated nursery course.The patient was admitted to the intensive care unit and received 15 U of cryoprecipitate, 2 U of fresh frozen plasma, and 2 U of red blood cells with continued hemodynamic instability requiring norepinephrine and vasopressin. She underwent an extensive hemolysis workup for inadequate transfusion response. Due to initial concern for postnatal thrombotic microangiopathy and thrombotic thrombocytopenia purpura, she received plasma exchange (PLEX) and Eculizumab. There was a rising suspicion of acute fatty liver of pregnancy (AFLP), given worsening hepatic dysfunction and encephalopathy. An ultrasound demonstrated a normal-sized liver with heterogeneous echogenicity and perihepatic/perisplenic ascites.Hepatology was consulted for the management of acute liver failure (ALF), and the patient was transferred to our transplant center. She was evaluated, listed, and received an orthotopic liver transplant (LT) from an HCV+ donor within 24 hours and was admitted to our transplant intensive care unit. She received continuous veno-venous hemofiltration with eventual renal recovery. Hemolytic workup revealed +direct Coombs test with +IgG consistent