HELLP syndrome

Background

• HELLP = Hemolysis, Elevated LFTS, Low Platelets
• Clinical variant of preeclampsia
• Any pt >20wk gestation or <4wk postpartum c/o abd pain should receive HELLP w/u
• Usually presents w/in 48 hr of delivery
• 80% of pts have no evidence of preeclampsia before delivery

Clinical Features

• RUQ or epigastric pain - 40-90%
• Proteinuria - 86-100%
• Hypertension - 82-88%

Differential Diagnosis

3rd Trimester/Postpartum Emergencies

• Acute fatty liver of pregnancy
• Amniotic fluid embolus
• Chorioamnionitis
• Eclampsia
• HELLP syndrome
• Mastitis
• Peripartum cardiomyopathy
• Postpartum endometritis (postpartum PID)
• Postpartum headache
• Postpartum hemorrhage
• Preeclampsia
• Resuscitative hysterotomy
• Retained products of conception
• Septic abortion
• Uterine rupture

Microangiopathic Hemolytic Anemia (MAHA)

• Disseminated Intravascular Coagulation (DIC)
• Thrombotic Thrombocytopenic Purpura (TTP)
• Hemolytic Uremic Syndrome (HUS)
• HELLP syndrome
• Heparin-Induced Thrombocytopenia (HIT)
• Hereditary spherocytosis
• Paroxysmal nocturnal hemoglobinuria (PNH)
• Malignant hypertension
• Scleroderma
• Antiphospholipid Syndrome (APS)
• Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
• Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
• Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM

Diagnosis

• CBC
  • Microangiopathic hemolytic anemia (Schistocytes)
  • Plt count <100
• Chemistry
  • Normal or elevated BUN/Cr
• Coags
  • Abnormal
• LFT
  • AST > 70, bilirubin > 1.2
  • ALT elevation (although usually <500)
• LDH > 600
• CT or US to check for hepatic hematoma (if needed)

Treatment

• Similar to that of severe preeclampsia or eclampsia
  • Delivery (only definitive treatment)
  • Magnesium
  • BP control
  • Coagulopathy correction
    • Transfuse platelets if < 20,000 or active, uncontrolled bleeding

Complications

• DIC
• Acute renal failure
• Subcapsular liver hematoma
  • Abdominal distention
  • Maintain adequate intravascular volume
    • If unstable consider embolization vs surgery

See Also

• Post-Partum Emergencies

References

• EBM, Volume 12, Number 8, Aug 2010. Postpartum Emergencies