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%)

Complications

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

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 <150 × 10⁹ per L (150 per mm³)
• Chemistry
  • Normal or elevated BUN/Cr
• Coagulation panel (fibrinogen can be decreased and PTT elevated)
  • Patients can progress towards DIC
• LFTs
  • AST levels > 150 IU/L and ALT levels > 100 IU/L (also often associated with bilirubin > 1.2mg/dL
  • LDH elevation (often > 600 IU/L)
• UA (proteinuria)
• 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

See Also

• Post-Partum Emergencies