Pregnancy-Associated Hemolysis,Elevated Liver Functions,Low Platelets (HELLP) Syndrome: An Obstetric Disease in the Intensive Care Unit

We reviewed the experience with hemolysis, elevated liver functions, low platelets (HELLP) syndrome at the University of New Mexico Hospital over the past 10 years to delineate the epidemiology and the clinical course of the disease. A retrospective chart review of a large, university-based, largely indigent, pregnant population was undertaken with attention to diagnostic features of the syndrome and maternal and infant outcomes of affected pregnancies. Comparisons were made with other large reported clinical series. Preeclampsia complicated 5.6% of deliveries in this study, and the HELLP syndrome occurred in 116 patients (0.3% of all deliveries). Epigastric pain, nausea, vomiting, and malaise usually heralded the syndrome. Signs and symptoms of preeclampsia (e.g., hypertension, proteinuria, and edema) were most often not striking at the time of presentation. The mean platelet count at diagnosis was 60 ± 25 × 10³. Initial signs of hemolysis were usually minimal, although the hematocrit decreased a mean of 11.5 ± 5 vol% over the course of the disease. Moderate elevations of hepatic enzyme levels, specifically lactic dehydrogenase, were common; hyperbilirubinemia was usually mild and late. Maternal complications included disseminated intravascular coagulation, eclampsia, pulmonary edema, adult respiratory distress syndrome, and hepatic hematoma. Eight stillbirths and 7 neonatal deaths occurred (perinatal mortality, 126/1,000). HELLP syndrome is a relatively infrequent but serious development in the evolution of preeclampsia/eclampsia. Variations in onset and severity of hemolysis, hepatic dysfunction, and thrombocytopenia are common. Maternal complications and the perinatal mortality rate are high. Pregnant patients with right upper quadrant or epigastric pain and thrombocytopenia, even in the absence of significant hypertension and proteinuria, are most likely to have HELLP syndrome, rather than some other medical condition. Awareness of the variable presentations of this syndrome permit the prompt recognition and management necessary to optimize maternal and neonatal outcomes.