Snapshot A 35-year-old G4P3 at 35 weeks gestation presents to the emergency room with nausea, vomiting, and right upper quadrant pain. She has a history of pre-eclampsia in her previous pregnancy. On physical exam, she is noted to be dry mucous membranes and mild periorbital edema. Her blood pressure is 180/90 mmHg. Laboratory testing shows elevated liver enzymes, increased lactate dehydrogenase, and decreased platelets. She is started on steroids, intravenous magnesium sulfate, and prepped for immediate delivery. Introduction Overview HELLP syndrome is named for its characteristic findings: Hemolysis, Elevated Liver enzymes, and Low Platelets it is a life-threatening complication of pregnancy some consider this a severe form or preeclampsia/eclampsia, though this is controversial Associated conditions preeclampsia and eclampsia Epidemiology Incidence 0.1-0.6% of all pregnancies Demographics usually occurs after 27 weeks of pregnancy can also occur postpartum, commonly 2 days after delivery Risk factors maternal age > 34 years multiparity Caucasian/European descent ETIOLOGY Pathogenesis mechanism thought to be due to endothelial activation, consumption of platelets, microangiopathic hemolysis, and microvascular injury complement dysfunction may also play a role Presentation Symptoms common symptoms nausea and vomiting right upper quadrant pain headache visual changes jaundice Physical exam inspection jaundice dry mucous membranes weakness hypertension nondependent edema periorbital upper or lower extremities Studies Serum labs elevated AST/ALT hemolysis elevated lactate dehydrogenase elevated bilirubin hemolysis on peripheral smear schistocytes, helmet cells, and burr cells decreased platelets typically normal coagulation studies Differential Acute fatty liver of pregnancy key distinguishing factor characterized primarily with fulminant liver failure Preeclampsia key distinguishing factor signs and symptoms of preeclampsia have significant overlap with HELLP syndrome however, preeclampsia classically occurs in younger patients (mean age of 19) and is characterized by hypertension and proteinuria Treatment Medical resuscitation indications all patients modalities intravenous fluids transfusion of blood products intravenous steroids indications platelets < 100,000/mm3 intravenous magnesium sulfate indications seizure prophylaxis antihypertensive medications indications hypertension drugs labetalol hydralazine nifedipine Surgical delivery indications if stable, consider steroids and immediate delivery if not stable, immediate delivery Complications Maternal complications cerebral hemorrhage disseminated intravascular coagulopathy acute renal failure hepatic rupture risk of recurrence in subsequent pregnancies increased risk of preeclampsia, preterm delivery, and placental abruption Neonatal complications prematurity intrauterine growth retardation fetal demise Other complications increased risk of cardiovascular disease Prognosis Most patients stabilize within 48 hours Maternal mortality 1-3%