HELLP syndrome

Background

• HELLP = Hemolysis, Elevated LFTs, Low Platelets
• Clinical variant of preeclampsia
• Any patient >20wk gestation or <4wk postpartum complaining of abdominal pain should receive HELLP workup
• When occurring postpartum, typically presents within 48hr of delivery
• 80% of patients have no evidence of preeclampsia before delivery

Clinical Features

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

Differential Diagnosis

Postpartum Emergencies

• Amniotic fluid embolus
• Chorioamnionitis
• Eclampsia
• Postpartum endometritis
• Postpartum headache
• HELLP syndrome
• Postpartum hemorrhage
• Mastitis
• Peripartum cardiomyopathy
• Preeclampsia
• Retained products of conception
• 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
• Malignant hypertension
• Antiphospholipid syndrome
• Scleroderma
• Antiphospholipid Syndrome (APS)
• Paroxysmal Nocturnal Hemoglobinuria (PNH)
• 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

Evaluation

Evaluation is targeted at assessing the individual components of the syndrome. Labs to be drawn should include a:

• CBC
  • May see a microangiopathic hemolytic anemia (Schistocytes on microscopy)
  • Platelet count <100/µL
• Chemistry
  • Normal or elevated BUN/Cr
• Coagulation panel
  • Abnormalities only if there is component of DIC
• LFT
  • AST > 70 U/L, bilirubin > 1.2mg/dL
  • ALT elevation (although usually <500)
• LDH > 600 IU/L
• CT or ultrasound can be considered if there is pain requiring a greater workup.
  • Depending on degree of thrombocytopenia are at risk for developing intraabdominal bleeding such as a hepatic hematoma

Management

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

Disposition

• Admit

Complications

• Disseminated intravascular coagulation
• Acute kidney injury
• Subcapsular liver hematoma
• Hemorrhage

See Also

• Post-Partum Emergencies

References

Authors

Ross Donaldson, Daniel Ostermayer, Michael Holtz, Amr Badawy, Claire, Neil Young, Jordan Swartz