What is the recommended management for a pregnant woman with HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelet count)?

HELLP Syndrome Management

HELLP syndrome is a manifestation of severe preeclampsia requiring immediate maternal stabilization followed by prompt delivery as the only definitive treatment, with delivery indicated at ≥34 weeks gestation or earlier if maternal or fetal condition deteriorates. 1, 2, 3

Immediate Maternal Stabilization

Seizure Prophylaxis

• Administer magnesium sulfate immediately to all women with HELLP syndrome and severe hypertension to prevent eclamptic seizures. 1, 2, 3
• Continue magnesium sulfate as a neuroprotective agent if delivery is required before 32 weeks gestation. 1, 3
• Maintain magnesium sulfate for 24 hours post-delivery to prevent eclampsia and pulmonary edema. 4

Blood Pressure Control

• For non-severe hypertension (140-159/90-109 mmHg): initiate oral labetalol, nifedipine, or methyldopa. 1, 3
• For severe hypertension (≥160/110 mmHg): treat urgently in a monitored setting with oral labetalol, nifedipine, or methyldopa; use intravenous labetalol or hydralazine if rapid control is needed. 1, 3, 4
• Target blood pressure below 155/105 mmHg. 5

Coagulopathy Management

• Platelet transfusion should be considered when platelet count is <100×10⁹/L due to increased risk of abnormal coagulation and adverse maternal outcomes. 1, 3
• Platelet transfusion is mandatory before cesarean section if platelets <50,000/mm³. 2, 3
• Administer fresh frozen plasma to correct coagulopathy as needed. 2

Critical Monitoring

• Transfer to ICU or high-dependency unit for continuous monitoring of blood pressure, central venous pressure, urinary output, ECG, and oxygen saturation. 2, 3
• Insert urinary catheter for hourly output monitoring (oliguria <400 mL/24h indicates severe disease). 2
• Consider central venous catheter or pulmonary artery catheter for fluid management, as these patients are often relatively hypovolemic. 2

Diagnostic Evaluation

Essential Laboratory Assessment

• Complete blood count with platelet count (platelet count <100,000/mm³ indicates severe thrombocytopenia and active HELLP syndrome). 2, 3
• Liver function tests including AST, ALT, LDH, and total bilirubin (elevated liver enzymes frequently correlate with adverse maternal outcomes). 1, 2
• Evaluation for hemolysis (serum haptoglobin is most sensitive for early recognition). 6
• Coagulation studies. 3
• Laboratory monitoring should continue every 6-12 hours for at least 24-48 hours postpartum, as 30% of HELLP cases occur or worsen postpartum. 2

Imaging for Hepatic Complications

• Perform abdominal imaging (ultrasound or CT) to rule out hepatic hemorrhage, infarct, or rupture, especially if right upper quadrant pain, epigastric pain, or right shoulder pain is present. 2, 3, 4
• 65% of symptomatic HELLP patients present with right upper quadrant or epigastric pain, which should immediately trigger imaging. 2
• Hepatic complications are more likely with platelet count <20×10⁹/L. 2

Timing and Mode of Delivery

Immediate Delivery Indications

• ≥34 weeks gestation: deliver immediately after maternal stabilization. 1, 2, 3
• Any gestational age with maternal deterioration (worsening laboratory values, severe symptoms, hepatic complications). 2
• Any gestational age with fetal compromise (non-reassuring fetal status, intrauterine growth restriction with abnormal Doppler). 2
• Hepatic hemorrhage, infarct, or rupture identified on imaging necessitates expeditious delivery after stabilization. 2

Expectant Management (<34 Weeks)

• Expectant management may be considered ONLY at <32-34 weeks with stable maternal condition (no worsening laboratory values, no severe symptoms), though this carries increased risk of adverse maternal outcomes. 2
• Conservative treatment beyond 48 hours is controversial and should only be attempted in select cases at perinatal centers with expertise. 7, 5

Mode of Delivery

• Vaginal delivery is preferable if obstetric conditions allow. 7, 5
• Expect high cesarean section rate (typically >70%) due to unfavorable cervix, fetal distress, and maternal deterioration. 2
• General anesthesia may be required if platelets <100,000/mm³ preclude regional anesthesia due to risk of epidural hematoma. 2

Corticosteroid Use

Maternal Treatment

• Corticosteroids should NOT be given to improve maternal outcomes in HELLP syndrome—they are ineffective for maternal disease. 1, 3, 4

Fetal Lung Maturation

• High-dose dexamethasone or betamethasone should be administered for fetal lung maturity if delivery is anticipated before 35 weeks gestation. 1, 3, 4
• Use either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg dexamethasone 12 hours apart. 5
• Avoid high-dose treatment and repeated doses due to potential long-term adverse effects on the fetal brain. 5

Critical Pitfalls to Avoid

• Do NOT delay delivery waiting for laboratory values to normalize—HELLP syndrome only resolves after delivery, and maternal mortality is 3.4% with delayed intervention. 2, 3
• Do NOT use regional anesthesia (epidural/spinal) with platelets <100,000/mm³ due to risk of epidural hematoma. 2
• Do NOT use corticosteroids to improve maternal HELLP outcomes. 1, 3, 4
• Do NOT use liver tests in isolation to guide care—they should be part of comprehensive maternal assessment. 1

Postpartum Management

• Continue monitoring in high-dependency or intensive care setting for at least 24-48 hours post-delivery. 2, 3
• Continue magnesium sulfate and strict fluid balance for 24 hours post-delivery to prevent pulmonary edema. 4
• Monitor for hepatic hemorrhage with abdominal ultrasound if symptoms occur postpartum. 4
• Liver function and platelet counts typically normalize within days to weeks after delivery. 3

Prevention in Future Pregnancies

• Women with history of prior HELLP syndrome should undergo first-trimester screening to assess risk of early-onset preeclampsia. 1, 3
• For high-risk women: initiate aspirin prophylaxis 150 mg nightly before 16 weeks gestation and continue until 36 weeks, delivery, or diagnosis of preeclampsia/HELLP. 1, 3, 4
• In women with low calcium intake (<800 mg/day), calcium supplementation (1.5-2 g elemental calcium/day) may reduce risk of both early and late-onset preeclampsia. 1, 3

Atypical Presentations

• HELLP syndrome can occur without hypertension in 15% of cases and without proteinuria in 5-15% of cases. 2, 6
• 30% of HELLP cases occur postpartum, with 20% occurring within 48 hours of delivery. 2, 6
• Partial HELLP syndrome (presence of any two components of the triad) is a progressive condition that can rapidly deteriorate and warrants intensive monitoring and preparation for delivery. 2