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A5166 - Early Empiric Plasmapheresis for Acute Fatty Liver of Pregnancy May Offer Benefit Even When the Diagnosis Is Unclear
Author Block: C. Oronce1, C. Yan1, M. Towner2, D. Nagel3, A. Pietropaoli1; 1Medicine, University of Rochester Medical Center, Rochester, NY, United States, 2Obstetrics and Gynecology, University of Rochester Medical Center, Rochester, NY, United States, 3Pulmonary and Critical Care, University of Rochester Medical Center, Rochester, NY, United States.
Introduction:
Acute fatty liver of pregnancy is a rare disorder that can be rapidly fatal to the mother and fetus. Diagnosis is often confounded because it can resemble pre-eclampsia; hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome; thrombotic thrombocytopenic purpura (TTP); or hemolytic uremic syndrome (HUS).
Case:
A 24-year-old healthy primigravid woman at 31 weeks gestation presented to an outside hospital with two days of abdominal discomfort, palpitations and vaginal bleeding. Her antepartum history included polyhydramnios, intrauterine growth restriction, and intraplacental clot. Initially, her AST and ALT were 170 and 162 respectively, and her creatinine was 2.04. She was transferred to a tertiary care center, and on arrival was tachycardic and oliguric. She underwent emergent Cesarean delivery due to fetal distress. Repeat labs revealed rising creatinine, elevated lactate, hypoglycemia, stable transaminases, severe hyperbilirubinemia, profoundly elevated PT/PTT, undetectable fibrinogen, hemolytic anemia and normal platelets. Following delivery, she experienced increased abdominal pain and nausea/vomiting; encephalopathy and asterixis were present on exam. Her liver enzymes improved, but she developed worsening renal failure, coagulopathy, hemolytic anemia and thrombocytopenia. Total cholesterol was 79 mg/dL with HDL 3 mg/dL. Work-up for infectious, autoimmune, metabolic, and toxic etiologies was unrevealing. Supportive treatment with continuous renal replacement therapy, blood products, and dextrose were administered. Empiric plasmapheresis was initiated one day later. Her mental status improved after three sessions, but ADAMTS13 activity was normal. Abdominal ultrasound one week later showed new hepatic steatosis. She achieved complete clinical recovery with normalization of labs after three months.
Discussion:
Acute fatty liver of pregnancy is a rare complication that may resemble pre-eclampsia, HELLP, or TTP/HUS. AFLP presents with encephalopathy, nausea/vomiting, and abdominal pain in the third trimester or postnatally. In addition to liver failure, laboratory studies may reveal low cholesterol, lactic acidosis, hypoglycemia, thrombocytopenia, disseminated intravascular coagulopathy, or kidney injury. Diagnosis is established by meeting six of the 14 Swansea criteria. Our patient met 11 of these clinical and laboratory criteria. Patients appearing to have the HELLP syndrome but with biochemical derangements and coagulopathy out of proportion to hepatic injury should be evaluated for AFLP. Early recognition with immediate delivery and supportive treatment in ICU settings portend better outcomes. Hepatic encephalopathy and lactic acidosis are the most predictive factors for death or transplantation. Importantly, even when the diagnosis remains ambiguous, early plasmapheresis for potential TTP/HUS should not be delayed. Our case adds to the growing literature that supports early plasmapheresis.