How should a pregnant woman beyond 20 weeks gestation presenting with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome) be managed?

Management of HELLP Syndrome

Immediate Actions Upon Diagnosis

Deliver the patient expeditiously after maternal stabilization, regardless of gestational age, as HELLP syndrome only resolves after delivery and delayed intervention carries a 3.4% maternal mortality risk. 1

Critical Initial Steps (First 30 Minutes)

• Initiate magnesium sulfate immediately for seizure prophylaxis in all women with HELLP syndrome, particularly those with severe hypertension 1
• Transfer to ICU or high-dependency unit for continuous monitoring of blood pressure, central venous pressure, urinary output, ECG, and oxygen saturation 1
• Obtain abdominal imaging (ultrasound or CT) immediately to rule out hepatic hemorrhage, infarct, or rupture—this is mandatory before proceeding to delivery 1
• Insert urinary catheter for hourly output monitoring; oliguria <400 mL/24h indicates severe disease 1
• Consider central venous catheter for fluid management, as these patients are often relatively hypovolemic despite appearing fluid overloaded 1

Laboratory Monitoring Protocol

• Check complete blood count, liver enzymes (AST, ALT, LDH), total bilirubin, coagulation profile (PT, aPTT, fibrinogen), and renal function immediately and repeat every 6-12 hours 1
• Platelet count <100,000/mm³ indicates severe thrombocytopenia and active HELLP syndrome with significant maternal risk 1
• Elevated liver enzymes (ALT, AST) frequently correlate with adverse maternal outcomes and indicate need for urgent intervention 1

Blood Pressure and Seizure Management

• Continue magnesium sulfate for 24 hours post-delivery to prevent eclamptic seizures 2
• For severe hypertension (≥160/110 mmHg): Use IV labetalol or hydralazine urgently in a monitored setting 2
• For non-severe hypertension: Use oral labetalol, nifedipine, or methyldopa 2

Correction of Coagulopathy Before Delivery

• Transfuse platelets if count <50,000/mm³ before any surgical intervention; strongly consider at higher levels given increased bleeding risk 1, 2
• Administer fresh frozen plasma to correct coagulopathy as needed 2
• Transfuse packed red blood cells if hemoglobin <10 g/dL 2

Timing and Mode of Delivery

Immediate Delivery Indications (After Stabilization)

• ≥34 weeks gestation: Deliver immediately after maternal stabilization 1
• Any gestational age with maternal deterioration: Worsening laboratory values, severe symptoms, or hepatic complications mandate immediate delivery 1
• Any gestational age with fetal compromise: Non-reassuring fetal status or IUGR with abnormal Doppler requires immediate delivery 1
• Hepatic hemorrhage, infarct, or rupture on imaging: Expeditious delivery after stabilization 1

Expectant Management (<34 Weeks)—Use With Extreme Caution

• Only consider expectant management at <32-34 weeks if the patient has stable maternal condition (no worsening laboratory values, no severe symptoms) 1
• This practice carries increased risk of adverse maternal outcomes and requires intensive monitoring 1
• Administer corticosteroids for fetal lung maturation (single course) if attempting expectant management 3

Mode of Delivery

• Vaginal delivery is preferable if cervical conditions are favorable and no maternal/fetal contraindications exist 3, 4
• Expect high cesarean section rate (>70%) due to unfavorable cervix, fetal distress, and maternal deterioration 1
• Regional anesthesia (epidural/spinal) is contraindicated if platelets <100,000/mm³ due to risk of epidural hematoma 1
• General anesthesia may be required if platelets preclude regional anesthesia 1

Postpartum Management (Critical 48-Hour Period)

HELLP syndrome typically peaks 24 hours after delivery; 30% of cases occur or worsen postpartum, requiring continued aggressive monitoring for at least 48 hours. 1, 2

Intensive Monitoring

• Continue laboratory monitoring every 6-12 hours for at least 24-48 hours postpartum 1
• Maintain continuous blood pressure, ECG, oxygen saturation, and central venous pressure monitoring 2
• Monitor hourly urinary output via indwelling catheter 2

Fluid Management

• Strict fluid balance is mandatory to avoid pulmonary edema for 24 hours after delivery 2
• Avoid aggressive fluid resuscitation that might reflexively be given in other critically ill patients, as this significantly increases pulmonary edema risk 2
• Administer fresh frozen plasma 400 mL cautiously to increase plasma volume, guided by CVP (target CVP 3-8 mmHg) 2

Complication Surveillance

• Monitor for hepatic hemorrhage or hematoma: Perform abdominal ultrasound if right upper quadrant pain, epigastric pain, or right shoulder pain develops 2
• Watch for acute renal failure: Oliguria <400 mL/24 hours indicates severe disease 2
• Monitor for pulmonary edema, DIC, and eclampsia 2
• Platelet count <20×10⁹/L correlates with higher risk of hepatic hemorrhage 2

Critical Pitfalls to Avoid

• DO NOT delay delivery waiting for laboratory values to normalize—HELLP only resolves after delivery 1
• DO NOT use regional anesthesia with platelets <100,000/mm³ due to epidural hematoma risk 1
• DO NOT administer corticosteroids to improve maternal HELLP outcomes—they are ineffective for maternal disease (only use for fetal lung maturation if <34 weeks) 1, 2
• DO NOT miss hepatic complications: 65% of symptomatic HELLP patients present with right upper quadrant or epigastric pain, which should immediately trigger imaging 1

Recognizing Atypical Presentations

• HELLP can occur with normal blood pressure in 15% of cases 1
• Absence of proteinuria does not rule out diagnosis 1
• 30% of cases present postpartum, with 20% occurring within 48 hours of delivery 1

Future Pregnancy Counseling

• Recurrence risk is 20% in future pregnancies 2
• Recommend aspirin prophylaxis 150 mg nightly starting before 16 weeks gestation until 36 weeks or delivery 2
• First-trimester screening for early-onset preeclampsia should be performed 2