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

Management of HELLP Syndrome

Deliver the patient promptly once maternal stabilization is achieved with blood pressure control and correction of coagulopathy, as HELLP syndrome only resolves after delivery. 1, 2

Initial Stabilization and Monitoring

Immediately initiate magnesium sulfate for seizure prophylaxis in all women with HELLP syndrome and severe hypertension. 2

• Transfer to ICU or high-dependency unit for continuous monitoring of blood pressure, central venous pressure, urinary output, ECG, and oxygen saturation 2
• Control blood pressure to keep it below 155/105 mmHg using IV magnesium sulfate or hydralazine 1, 3
• Insert urinary catheter for hourly output monitoring (oliguria <400 mL/24h indicates severe disease) 1, 2
• Consider central venous catheter in critically ill patients, as these patients are often relatively hypovolemic 1, 2
• Maintain strict fluid balance to avoid pulmonary edema, which is a critical complication 4

Comprehensive Laboratory Assessment

Perform immediate laboratory investigations including: 1

• Platelet count, white blood cell count, PCV, partial thromboplastin time
• Fibrinogen concentration, fibrin degradation products, peripheral blood smear
• Liver function tests (AST, ALT, LDH, total bilirubin are most useful for following disease progression) 2
• Creatinine, urea, and uric acid levels
• Chest X-ray to exclude pulmonary edema and ECG 1

A platelet count below 100,000/mm³ indicates severe thrombocytopenia and active HELLP syndrome with significant maternal risk. 2

Blood Product Transfusion Thresholds

Transfuse platelets if count is less than 50,000/mm³, especially if cesarean delivery is planned. 1, 2, 4 This is mandatory before any surgical intervention. 2

• Transfuse whole blood or packed red cells if hemoglobin is less than 10 g/dL 1, 4
• The degree of thrombocytopenia correlates directly with severity of liver dysfunction and predicts adverse maternal outcomes 2

Timing and Indications for Delivery

Delivery is indicated in the following scenarios: 1

• Gestational age at or beyond 32-34 weeks
• Worsening pre-eclampsia or deteriorating hepatic or renal function
• Severe thrombocytopenia (especially <20×10⁹/L, which correlates with higher risk of hepatic hemorrhage) 4
• Evidence of fetal distress or fetal maturity
• Any maternal condition deterioration

Between 24-34 weeks gestation, 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) before delivery. 3, 5 However, corticosteroids are ineffective for improving maternal HELLP outcomes and should not be given for this purpose. 2, 4

Conservative management beyond 48 hours is controversial and may only be considered in highly selected cases <34 weeks' gestation with stable maternal and fetal conditions. 3, 5

Mode of Delivery

• Vaginal delivery is preferable when feasible 3, 5
• Expect high cesarean section rates (61.5-76%) due to obstetric indications 1, 2
• If cervix is unfavorable, induce cervical ripening then labor 3

Anesthetic Considerations

Regional anesthesia (epidural/spinal) is contraindicated with platelets <100,000/mm³ due to risk of epidural hematoma. 2

• If general anesthesia is required, use propofol (minimal hepatic or renal metabolism) 1
• Consider rapid sequence induction with suxamethonium for airway management 1

Critical Imaging for Hepatic Complications

Perform abdominal imaging (ultrasound or CT) immediately in patients with right upper quadrant or epigastric pain to rule out subcapsular hematoma or hepatic rupture. 2

• 65% of symptomatic HELLP patients present with right upper quadrant or epigastric pain 2
• Hepatic rupture requires expeditious delivery after maternal stabilization, regardless of gestational age 2
• Early referral to transplant center is necessary if signs of hepatic failure develop 4

Post-Delivery Management

Continue intensive monitoring for at least 48 hours postpartum, as 30% of HELLP cases occur or worsen postpartum. 2, 3, 5

• Continue magnesium sulfate and strict fluid balance for 24 hours after delivery 4
• Perform serial laboratory monitoring every 6-12 hours for at least 24-48 hours postpartum 2
• Monitor for complications including hemorrhage, renal failure, pulmonary edema, and hepatic hematoma 1, 4
• Monitor for hypoglycemia during management 1, 4

Critical Pitfalls to Avoid

Do not delay delivery waiting for laboratory values to normalize—maternal mortality is 3.4% with delayed intervention. 2

• Avoid aggressive fluid resuscitation that might reflexively be given in other critically ill patients, as this significantly increases pulmonary edema risk 4
• Do not use repeated or high-dose corticosteroids for maternal benefit, as they are ineffective and may cause long-term adverse fetal brain effects 3
• Do not miss delayed diagnosis—symptoms may mimic acute cholecystitis, drug reactions, or idiopathic thrombocytopenia 1, 4

Future Pregnancy Counseling

• Counsel regarding increased risk of recurrence in future pregnancies 4
• Consider aspirin prophylaxis in future pregnancies starting before 16 weeks' gestation 4