What is the management of HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Management of HELLP Syndrome

The definitive treatment for HELLP syndrome is delivery of the fetus, as this is the only intervention that resolves the condition and reduces maternal morbidity and mortality, which can be as high as 3.4% if left untreated. 1

Diagnosis and Initial Assessment

• HELLP syndrome is characterized by hemolysis, elevated liver enzymes, and low platelet count, typically occurring as a severe variant of pre-eclampsia in 10-20% of cases with severe preeclampsia 1, 2
• Clinical presentation includes epigastric pain, upper abdominal tenderness, hypertension, proteinuria, nausea, vomiting, and in some cases jaundice 1
• Laboratory criteria include:
  • Evidence of hemolysis with increased LDH (>600 U/L)
  • Elevated liver enzymes (AST ≥70 U/L)
  • Low platelet count (<100,000/mm³) 1, 2
• About 70% of cases develop before delivery, primarily between 27-37 weeks gestation, with the remainder occurring within 48 hours after delivery 2

Initial Stabilization

• Insert urinary catheter for hourly monitoring of output to assess renal function 1
• Consider central venous catheter placement in critically ill patients for fluid management and monitoring 1
• Control blood pressure with IV medications:
  • Magnesium sulfate for seizure prophylaxis
  • Hydralazine to maintain blood pressure below 155/105 mmHg 1, 2
• Perform comprehensive laboratory investigations:
  • Complete blood count with peripheral smear
  • Coagulation studies (PT, PTT, fibrinogen, fibrin degradation products)
  • Liver function tests
  • Renal function tests 1

Blood Product Management

• Administer platelet transfusion if count is less than 50,000/mm³, especially if delivery by cesarean section is planned 1
• Transfuse whole blood or packed red blood cells if hemoglobin concentration is less than 10 g/dL 1
• Consider fresh frozen plasma to correct hypovolemia and coagulopathy 3

Definitive Treatment

• Indications for immediate delivery include:

  • Worsening pre-eclampsia
  • Deteriorating hepatic or renal function
  • Severe thrombocytopenia
  • Gestational age at or beyond 32-34 weeks
  • Evidence of fetal distress
  • Evidence of fetal maturity 1, 2

• For gestations <34 weeks:

  • Administer a single course of corticosteroids for fetal lung maturation (2 doses of 12 mg betamethasone 24 hours apart or 6 mg dexamethasone 12 hours apart) 1, 2
  • Conservative management (>48 hours) is controversial but may be considered in selected cases <34 weeks if maternal and fetal conditions remain stable 2
  • Proceed with delivery if maternal condition worsens or signs of fetal distress occur 2

• Mode of delivery:

  • Vaginal delivery is preferable when possible 2
  • If cervix is unfavorable, cervical ripening followed by induction of labor is reasonable 2
  • Cesarean section rates are high (61.5-76%) due to obstetric indications and maternal/fetal deterioration 1

Anesthetic Considerations

• The presence of liver dysfunction and severe thrombocytopenia governs the choice of anesthetic 3
• If general anesthesia is required, use drugs with minimal hepatic or renal metabolism (propofol is recommended) 1
• Consider rapid sequence induction with suxamethonium for airway management 1
• Regional anesthesia may be contraindicated with severe thrombocytopenia (platelets <50,000/mm³) 1

Post-Delivery Management

• Continue close surveillance of the mother for at least 48 hours after delivery 2
• Monitor for complications including:
  • Hemorrhage
  • Renal failure
  • Pulmonary edema 1
• Resolution of laboratory abnormalities typically follows a protracted course over several days after delivery 4
• Continue blood pressure control and fluid management until clinical and laboratory parameters normalize 1, 2

Special Considerations and Pitfalls

• Delayed diagnosis is common as symptoms may mimic other conditions such as acute cholecystitis, drug reactions, or idiopathic thrombocytopenia 1
• HELLP syndrome must be differentiated from other microangiopathic hemolytic anemias such as hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and acute fatty liver of pregnancy 5
• Hypoglycemia can occur and should be monitored during management 1
• The use of high-dose corticosteroids for maternal treatment remains controversial; while some studies suggest benefit 6, standard corticosteroid treatment is of uncertain clinical value for maternal HELLP syndrome 1, 2
• Emerging therapies such as complement inhibitors (Eculizumab) have shown promise in case reports for severe preeclampsia/HELLP syndrome but require further research 7