Acute Fatty Liver and HELLP Syndrome: Two Distinct Pregnancy Disorders

Abstract

Acute fatty liver of pregnancy (AFLP) has long been recognized as a clinical entity; early diagnosis and prompt delivery have much improved the outlook. Another hepatic disorder, found in varying proportions of women withpreeclampsia/eclampsia, is the HELLP syndrome of liver dysfunction, thrombocytopenia, and hemolytic anemia (hemolysis, elevated liver function test, low platelets). To clarify the relationship between these conditions, the investigators contrasted the findings in 75 consecutive cases of HELLP syndrome and 10 consecutive women with AFLP seen during the same period. Criteria for HELLP syndrome included preeclampsia, hemolysis with decreased hemoglobin, elevated liver enzymes, and a platelet count of 100,000 ug/liter or lower. A diagnosis of AFLP required clinically evident acute hepatic dysfunction in the third trimester that resolved completely postpartum. Maternal and gestational ages were comparable in the two groups, but women with HELLP syndrome had greater parity. Women with AFLP very often presented with malaise, nausea/vomiting, abdominal pain, and jaundice, whereas the picture of HELLP syndrome featured headache, abdominal or epigastric pain, and hematuria. AFLP patients tended to have low fibrinogen levels, and most of them had leukocytosis (not associated with sepsis). Antithrombin III levels were extremely low, and hypoglycemia was a regular finding. Total bilirubin levels averaged 11 and 1.4 mg in the AFLP and HELLP syndrome groups, respectively. Women with AFLP had very low cholesterol and triglyceride levels. AFLP was associated with significantly more serious maternal complications, including renal insufficiency, disseminated intravascular coagulation, encephalopathy, and sepsis. One fifth of patients with HELLP syndrome were in acute renal failure, and nearly as many had abruptio placentae. Significantly more AFLP patients required transfusions. Birth weights were somewhat lower in the HELLP group, though not significantly so. Two of 10 women with AFLP died after intestinal perforation and sepsis. There were two deaths among the 75 women with HELLP syndrome. The respective mean maternal hospital stays were 13 and 7 days. This comparative study documented significantly disparate symptoms, laboratory findings, and complications between women with AFLP and those with HELLP syndrome, implying pathophysiological differences.