HELLP syndrome

Background

• HELLP = Hemolysis, Elevated LFTS, Low Platelets
• Clinical variant of preeclampsia
• Any patient >20wk gestation or <4wk postpartum c/o abd pain should receive HELLP w/u
• Usually presents w/in 48 hr 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

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

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 > 1.2 mg/dL
  • ALT elevation (although usually <500)
• LDH > 600 IU/L
• CT or US can be considered if there is pain requiring a greater workup.
  • Patients depending on degree of thrombocytopenia are at risk of developming intraabdominal bleeding such as a hepatic hematoma

Management

• 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
• Hemorrhage

See Also

• Post-Partum Emergencies

References

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