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

Management of HELLP Syndrome

Deliver the patient as soon as maternal coagulopathy is corrected and severe hypertension is controlled—HELLP syndrome only resolves after delivery, and delaying definitive treatment increases maternal mortality risk. 1, 2

Initial Stabilization and Monitoring

Immediately initiate magnesium sulfate for seizure prophylaxis in all women with HELLP syndrome and severe hypertension. 2 This should be started before delivery and continued for 24 hours postpartum. 3

Blood Pressure Control

• Target blood pressure below 155/105 mmHg using IV magnesium sulfate or hydralazine. 1, 4
• For severe hypertension (≥160/90 mmHg), use IV labetalol or hydralazine in a monitored setting. 3
• An infusion of hydralazine can be commenced to reduce mean arterial pressure and avoid eclampsia or intracranial hemorrhage. 5

Invasive Monitoring

• 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 despite appearing edematous. 5, 1, 2
• Transfer to ICU or high-dependency unit for continuous monitoring of blood pressure, central venous pressure, oxygen saturation, and ECG. 2, 3

Laboratory Assessment and Blood Product Management

Essential Laboratory Tests

• Complete blood count with peripheral smear to assess for schistocytes and hemolysis. 1, 6
• Platelet count, liver function tests (AST, ALT, LDH, total bilirubin), and coagulation profile (PT, aPTT, fibrinogen, fibrin degradation products). 1, 2
• Renal function tests (creatinine, urea, uric acid). 1
• Chest X-ray to exclude pulmonary edema and ECG. 1

Blood Product Transfusion Thresholds

Transfuse platelets if count is <50,000/mm³, especially before cesarean section. 1, 2, 3 The degree of thrombocytopenia correlates directly with severity of liver dysfunction and predicts adverse maternal outcomes. 2

• Transfuse whole blood or packed red cells if hemoglobin is <10 g/dL. 1, 3
• Fresh frozen plasma 400 mL can be given to increase plasma volume if patient is hypovolemic. 5

Timing and Mode of Delivery

Indications for Immediate Delivery

Proceed with delivery if any of the following are present: 1

• Gestational age ≥32-34 weeks
• Worsening pre-eclampsia or deteriorating hepatic/renal function
• Severe thrombocytopenia (platelets <50,000/mm³)
• Evidence of fetal distress or fetal maturity
• Maternal symptoms including right upper quadrant or epigastric pain (65% of symptomatic patients—this should trigger imaging to rule out hepatic rupture). 2

Conservative Management <34 Weeks

• Conservative management (≥48 hours) may be considered in highly selected cases <34 weeks' gestation without maternal or fetal deterioration. 4, 7
• Administer single course of corticosteroids for fetal lung maturation: either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg dexamethasone 12 hours apart. 4
• Do NOT use corticosteroids to improve maternal HELLP outcomes—they are ineffective for maternal disease. 2, 3

Mode of Delivery

• Vaginal delivery is preferable if cervical conditions are favorable. 4, 7
• Cesarean section rates are high (61.5-76%) due to obstetric indications. 1, 2

Anesthetic Considerations

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

For general anesthesia: 1

• Use drugs with minimal hepatic or renal metabolism (propofol is recommended)
• Rapid sequence induction with thiopentone 5 mg/kg and suxamethonium 1.5 mg/kg 5, 1
• Fentanyl 5 µg/kg IV to attenuate hypertensive response to intubation 5

Postpartum Management

Immediate Post-Delivery Care

• Continue magnesium sulfate and strict fluid balance for 24 hours after delivery. 3
• Avoid aggressive fluid resuscitation—these patients require strict fluid balance to prevent pulmonary edema. 3
• Continue intensive monitoring in high-dependency unit for at least 48 hours postpartum. 4

Laboratory Monitoring

• Serial complete blood counts and liver function tests every 6-12 hours for at least 24-48 hours postpartum, as 30% of HELLP cases occur or worsen postpartum. 2, 3
• The intensity of HELLP syndrome typically peaks 24 hours after delivery. 6
• LDH and platelet count are the two best tests to monitor disease course. 6

Surveillance for Complications

Monitor for: 3

• Hepatic hemorrhage or hematoma (abdominal pain, epigastric pain, right shoulder pain)
• Renal dysfunction (oliguria, rising creatinine)
• Pulmonary edema (respiratory distress, oxygen desaturation)
• Coagulopathy (markedly reduced platelet count <20×10⁹/L correlates with higher risk of hepatic hemorrhage)
• Hypoglycemia 1, 3

Imaging for Hepatic Complications

Perform abdominal ultrasound or CT if right upper quadrant/epigastric pain persists to rule out subcapsular hematoma or hepatic rupture. 2 Fibrin deposition within hepatic sinusoids causes a predictable sequence of subcapsular hematomas, parenchymal hemorrhage, and potentially hepatic rupture. 2

Critical Pitfalls to Avoid

• Never delay delivery waiting for laboratory values to normalize—maternal mortality is 3.4% with delayed intervention. 2
• Never use regional anesthesia with platelets <100,000/mm³. 2
• Never give corticosteroids to improve maternal HELLP outcomes. 2, 3
• Never aggressively fluid resuscitate—this significantly increases pulmonary edema risk. 3
• Recognize that delayed diagnosis is common as symptoms may mimic acute cholecystitis, drug reactions, or idiopathic thrombocytopenia. 1, 3

Long-Term Counseling

• Counsel regarding increased risk of recurrence in future pregnancies (though recurrences are not frequent). 3, 7
• Consider aspirin prophylaxis in future pregnancies starting before 16 weeks' gestation. 3