The HELLP syndrome refers to the constellation of Hemolysis, Elevated Liver function tests and Low Platelet count seen in pregnant women and sometimes considered to be a severe form of eclampsia.

Epidemiology

• Incidence - 4-5/1000 pregnancies
  • 5-10% of pregnancies with preeclampsia
  • 30-50% of pregnancies with eclampsia
• Age - childbearing years
• Gender - exclusively in pregnant or postpartum females

Risk Factors (increased risk)

• Ethnic - Caucasian
• Multiparity
• Age - >34 years
• Presence of preeclampsia or eclampsia

Pathophysiology

• Placenta in preeclampsia is poorly perfused, which releases factors that create endothelial dysfunction
• Endothelial dysfunction causes platelet aggregation and altered ratio of thromboxane to prostacyclin
• Thrombin-induced activation of the coagulation cascades leads to hemolytic anemia and multiorgan microvascular injury

Clinical Presentation

• One or more of the following symptoms may be present
  • 2/3 of patients are diagnosed antepartum
• Nonspecific - malaise, fatigue
• Gastrointestinal - right upper quadrant pain, nausea, emesis
  • May experience subcapsular hematomas and liver rupture
• Central nervous system - headache, confusion
• Cardiovascular - hypertension

Treatment

• Delivery of fetus
• Glucocorticosteroid therapy
  • Nonrandomized studies suggest that they may improve outcomes

Diagnosis

• Laboratory testing
  • Peripheral smear - helmet cells, burr cells, schistocytes (all signs of microangiopathic hemolysis)
  • CBC - anemia, thrombocytopenia (<100,000/mm3)
  • LDH - markedly elevated (greater than or equal to 600 U/L)
  • Liver function tests - elevated
    • AST 2 times the normal value
• Patients without all of the criteria can be deemed partial HELLP

Differential Diagnosis

• Thrombotic thrombocytopenic purpura (TTP)
• Hemolytic uremic syndrome
• Disseminated intravascular coagulation (DIC)
• Antiphospholipid syndrome
• Acute fatty liver of pregnancy
• Viral hepatitis