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

Management of HELLP Syndrome

HELLP syndrome requires immediate maternal stabilization followed by prompt delivery, as this is the only definitive treatment that reduces maternal mortality and morbidity. 1, 2

Initial Stabilization and Assessment

Blood Pressure Control

• Initiate IV magnesium sulfate immediately to prevent eclamptic seizures and provide neuroprotection 2
• Control severe hypertension (>160/105 mmHg) with IV labetalol or hydralazine to prevent intracranial hemorrhage and eclampsia 3, 2
• Target mean arterial pressure below 105 mmHg 3

Essential Laboratory Investigations

Obtain comprehensive workup including: 3, 1

• Platelet count, white blood cell count, PCV
• Partial thromboplastin time, fibrinogen concentration, fibrin degradation products
• Peripheral blood smear for hemolysis confirmation
• Liver function tests (AST, ALT, LDH)
• Serum creatinine, urea, and uric acid
• Chest X-ray to exclude pulmonary edema
• ECG

Hemodynamic Monitoring

• Insert urinary catheter for hourly output monitoring (oliguria <400 ml/24h indicates severe disease) 3, 1
• Consider central venous catheter in critically ill patients for fluid management and volume assessment 3, 1
• Maintain strict fluid balance to avoid pulmonary edema—this is critical and differs from standard resuscitation protocols 2

Blood Product Management

Platelet Transfusion

• Transfuse platelets if count <50,000/mm³, especially before cesarean section 3, 1, 2
• Administer 6 units to achieve target platelet count >50,000/mm³ 3

Red Blood Cell Transfusion

• Transfuse whole blood or packed red cells if hemoglobin <10 g/dL 3, 2

Fresh Frozen Plasma

• Administer FFP for volume expansion and coagulopathy correction as needed 3

Timing and Mode of Delivery

Indications for Immediate Delivery

Proceed with delivery if any of the following are present: 3, 1

• Gestational age ≥32-34 weeks
• Worsening pre-eclampsia (increasing blood pressure, cerebral symptoms)
• Deteriorating hepatic or renal function
• Severe or progressive thrombocytopenia
• Evidence of fetal distress
• Evidence of fetal maturity

Conservative Management (<34 Weeks)

• May consider expectant management for 48 hours in highly selected cases <34 weeks gestation in a tertiary perinatal center 4, 5
• Administer single course of corticosteroids for fetal lung maturation: either betamethasone 12 mg IM x2 doses 24 hours apart OR dexamethasone 6 mg IM x4 doses 12 hours apart 4
• Do NOT use corticosteroids to improve maternal HELLP outcomes—the American College of Obstetricians and Gynecologists advises against this practice 2

Mode of Delivery

• Vaginal delivery is preferable if feasible 3, 4
• Cesarean section rates are high (61.5-76%) due to obstetric indications and maternal/fetal instability 3, 1

Anesthetic Considerations

Neuraxial vs General Anesthesia

• Spinal anesthesia is first choice if platelets are adequate and not rapidly declining 6
• General anesthesia is required if: 6
  • Severe thrombocytopenia (<50,000/mm³)
  • Rapidly progressive coagulopathy
  • Neurological signs present
  • Emergency delivery needed

General Anesthesia Technique

If general anesthesia is necessary: 3, 1

• Use propofol (minimal hepatic/renal metabolism preferred over thiopentone) 1
• Rapid sequence induction with suxamethonium 1.5 mg/kg IV 3
• Administer fentanyl 5 µg/kg IV to attenuate hypertensive response to intubation 3
• Maintain with 50% nitrous oxide and 0.5% isoflurane in oxygen 3
• Anticipate difficult airway—increased risk due to airway edema 6

Post-Delivery Management

Intensive Monitoring (First 24-48 Hours)

Transfer to high-dependency unit with continuous monitoring of: 3, 2

• Blood pressure (non-invasive or arterial line)
• Central venous pressure
• Hourly urinary output
• ECG and oxygen saturation

Continued Medical Management

• Continue magnesium sulfate for 24 hours post-delivery 2
• Maintain strict fluid balance for 24 hours to prevent pulmonary edema 2
• Continue antihypertensive therapy; wean hydralazine when blood pressure stabilizes (typically after 6 hours) 3

Serial Laboratory Monitoring

Monitor trends in: 2

• Platelet count (typically normalizes by day 2-3 post-delivery) 3
• Liver enzymes (normalize over following week) 3
• Hemoglobin and coagulation profile
• Renal function tests

Surveillance for Complications

Watch for signs of: 2

• Hepatic hemorrhage or hematoma (abdominal pain, right shoulder pain, epigastric pain)
• Renal failure (oliguria, rising creatinine)
• Pulmonary edema
• DIC
• Hypoglycemia 1, 2

Critical pitfall: Maternal mortality is 3.4%, and the condition can progress rapidly even postpartum 3, 1. Up to 30% of cases present postpartum, so maintain high vigilance for 48 hours after delivery 7, 5.

Long-Term Counseling

• Recurrence risk in future pregnancies ranges from 27-48% 7
• Consider aspirin prophylaxis in future pregnancies starting before 16 weeks' gestation 2