What is HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome in pregnancy?

HELLP Syndrome in Pregnancy

HELLP syndrome is a severe complication of pregnancy characterized by Hemolysis, Elevated Liver enzymes, and Low Platelets, occurring in 0.5-0.9% of all pregnancies and in 10-20% of cases with severe preeclampsia. 1, 2

Definition and Classification

HELLP syndrome is considered part of the spectrum of preeclampsia and not a separate disorder. According to the International Society for the Study of Hypertension in Pregnancy (ISSHP), women with features of HELLP syndrome should be considered to have preeclampsia so that all other features of preeclampsia will be sought and addressed. 1

Diagnostic criteria for HELLP syndrome include:

• Hemolysis with increased LDH (> 600 U/L)
• Elevated liver enzymes: AST ≥ 70 U/L
• Low platelet count: < 100 × 10^9/L 2

The Mississippi Triple-class HELLP System further classifies the disorder by the nadir platelet counts:

• Class 1: platelets ≤ 50,000/μL
• Class 2: platelets > 50,000/μL and ≤ 100,000/μL
• Class 3: platelets > 100,000/μL and ≤ 150,000/μL 2

Clinical Presentation

HELLP syndrome typically presents with:

• Epigastric or right upper quadrant pain (common presenting symptom)
• Nausea and vomiting
• Malaise or fatigue
• Headache
• Visual changes
• Hypertension (may not always be severe)
• Proteinuria (present in most but not all cases) 1, 2, 3

About 70% of cases develop before delivery, primarily between the 27th and 37th gestational weeks, while the remainder occur within 48 hours after delivery. 2, 4

Complications

HELLP syndrome is a progressive condition with serious maternal and fetal complications:

Maternal complications:

• Disseminated intravascular coagulation (DIC)
• Placental abruption
• Acute renal failure
• Pulmonary edema
• Adult respiratory distress syndrome
• Subcapsular liver hematoma or rupture
• Retinal detachment
• Cerebral hemorrhage
• Maternal death (mortality rate up to 24%) 2, 3

Fetal/neonatal complications:

• Prematurity
• Intrauterine growth restriction
• Perinatal mortality (79-367 per 1,000 live births) 3

Management

Diagnosis and Monitoring

All women with suspected preeclampsia or HELLP syndrome should undergo:

• Complete blood count with platelet count
• Liver function tests (AST, ALT, LDH)
• Renal function tests
• Coagulation studies
• Urinalysis and protein quantification 5

Treatment Approach

1. Delivery is the definitive treatment for HELLP syndrome, especially:

   • After 34 weeks' gestation
   • When maternal or fetal condition deteriorates 5, 2

2. Blood pressure control:

   • For severe hypertension (≥160/110 mmHg): IV labetalol, hydralazine, or nicardipine
   • For non-severe hypertension: oral labetalol, nifedipine, or methyldopa 5

3. Seizure prophylaxis:

   • Magnesium sulfate: loading dose 4-5g IV over 15-20 minutes, followed by maintenance dose of 1-2g/hour continuous infusion for at least 24 hours postpartum 5

4. Platelet management:

   • Platelet transfusion recommended for counts <100×10^9/L
   • Higher vigilance for hepatic hemorrhage with platelet counts <20×10^9/L 5

5. Corticosteroid therapy:

   • For fetal lung maturation between 24-34 weeks: single course of betamethasone (2 doses of 12 mg 24 hours apart) or dexamethasone (6 mg 12 hours apart)
   • Standard corticosteroid treatment for maternal HELLP syndrome is of uncertain clinical value 5, 2

Mode of Delivery

• Vaginal delivery is preferable when possible
• If cervix is unfavorable, cervical ripening followed by induction of labor is reasonable
• Cesarean section is indicated for standard obstetric indications or when rapid delivery is necessary due to deteriorating maternal or fetal condition 5, 2

Postpartum Care

• Close surveillance should continue for at least 48 hours after delivery
• Intensive care admission for severe cases with end-organ dysfunction
• Antihypertensive medications should be continued postpartum and tapered slowly after days 3-6
• Monitor blood pressure at least every 4-6 hours for at least 3 days postpartum 5, 4

Long-term Follow-up and Prevention

• Review at 3 months postpartum to ensure resolution of hypertension, proteinuria, and laboratory abnormalities
• Annual medical review recommended lifelong due to increased cardiovascular risk
• In subsequent pregnancies:
  • Low-dose aspirin (150 mg) before 16 weeks' gestation until 36 weeks
  • Calcium supplementation (1.2-2.5 g/day) if dietary intake is likely low 5

Risk of Recurrence

Women with a history of HELLP syndrome have an increased risk of developing hypertensive disorders in subsequent pregnancies and should receive extra monitoring throughout any future pregnancies. 1, 5