HELLP Syndrome

HELLP Syndrome

 

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

Diagnosis

  • Laboratory testing
    • Peripheral smear – helmet cells, burr cells, schistocytes (all signs of microangiopathic hemolysis)
    • CBC – anemia, thrombocytopenia (<100,000/mm3)
    • LDH – markedly elevated (≥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

Treatment

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

See Also