HELLP syndrome

Introduction

Hemolysis, Elevated Liver function tests, Low Platelets.

Epidemiology

• 10% of patients with pre-eclamsia develop HELLP syndrome

Pathology

• variant of pre-eclampsia
• microangiopathic hemolytic anemia
• may be difficult to distinguish from TTP/HUS
• causes significant maternal & fetal morbidity & mortality
• hepatic infarctions, hematomas & rupture may occur
  • liver biopsy shows necrosis, fibrosis, steatosis, hemorrhage
• fulminant hepatic failure may occur

Clinical manifestations

• occurs only occurs during 3rd trimester of pregnancy
• features of pre-eclampsia
• mild hypertension
• right upper quadrant pain, nausea
• disseminated intravascular coagulation usually present

Laboratory

• complete blood count (CBC) with peripheral smear
  • microangiopathic hemolytic anemia
  • thrombocytopenia
• liver function tests
  • elevated serum aminotransferases
    • serum ALT (< 500 U/L)
    • serum AST (< 500 U/L)
  • serum: bilirubin:
    • mild hyperbilirubinemia (2-3 mg/dL)
    • elevated serum conjugated bilirubin
• serum lactate dehydrogenase is elevated
• renal function tests
  • blood urea nitrogen: uremia
  • 24 hour urine volume: oliguria
  • 24 hour urine protein: proteinuria
    • 6% of patients without proteinuria
• prothrombin time (PT) & aPTT are normal
• plasma D-dimer usually elevated
• serum glucose may be low
• see ARUP consult

Differential diagnosis

• intrahepatic cholestasis of pregnancy
• acute fatty liver of pregnancy
• microangiopathic hemolytic distinguishes HELLP syndrome

Management

• immediate delivery of infant
• generally resolves 48 hours after delivery (resolution within days to weeks)

More general terms

• liver disease during pregnancy
• pregnancy-induced hypertension; gestational hypertension (PIH)
• thrombotic microangiopathy
• thrombocytopenia
• syndrome

Additional terms

• hemolytic uremic syndrome (HUS)
• pre-eclampsia/eclampsia
• thrombotic thrombocytopenic purpura (TTP)