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

Management of HELLP Syndrome

The definitive treatment for HELLP syndrome is immediate delivery regardless of gestational age, with platelet transfusion recommended if platelet count is less than 50,000/mm³, especially prior to Caesarean section. 1

Diagnosis and Initial Assessment

• HELLP syndrome is characterized by:
  • Hemolysis (with increased LDH >600 U/L)
  • Elevated liver enzymes (AST ≥70 U/L)
  • Low platelet count (<100 x 10⁹/L) 2
• HELLP represents a serious complication occurring in 10-20% of cases with severe preeclampsia 2
• About 70% of cases develop before delivery (majority between 27-37 weeks gestation), with the remainder occurring within 48 hours after delivery 2

Management Algorithm

Immediate Management

1. Maternal stabilization:

   • Control blood pressure: Target diastolic BP of 85 mmHg (systolic 110-140 mmHg)
   • First-line IV medications: Labetalol, Hydralazine, or oral Nifedipine 1
   • Administer magnesium sulfate for seizure prophylaxis, especially with neurological signs/symptoms 1

2. Laboratory monitoring:

   • Complete blood count with platelets
   • Liver function tests
   • Renal function tests
   • Coagulation studies
   • Continuous monitoring is essential as HELLP is a progressive condition 2

3. Delivery planning:

   • Immediate delivery is indicated regardless of gestational age as the definitive treatment 1
   • Mode of delivery depends on maternal condition, fetal status, and gestational age
   • Vaginal delivery is preferable if the maternal and fetal conditions allow 2
   • If cervix is unfavorable, cervical ripening followed by induction is reasonable 1

Specific Management Based on Gestational Age

1. If <34 weeks gestation:

   • Administer a 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 1, 2
   • Note: Standard corticosteroid treatment for maternal HELLP syndrome itself has uncertain clinical value 2

2. If ≥34 weeks gestation:

   • Proceed with immediate delivery 2

Blood Product Management

• Platelet transfusion if count <50,000/mm³, especially before Caesarean section 1
• Whole blood or packed red cells if hemoglobin <10 g/dL 1
• Consider fresh frozen plasma if there is evidence of coagulopathy

Postpartum Management

• Continue close surveillance for at least 48 hours after delivery 2
• Monitor blood pressure every 4-6 hours for at least 3 days 1
• Continue laboratory monitoring until parameters show clear improvement
• Taper antihypertensive medications slowly after days 3-6 1
• Follow-up within 1 week if still on antihypertensives at discharge 1

Special Considerations and Complications

• HELLP syndrome can be complete or incomplete, with varying severity 2
• The Mississippi Triple-class HELLP System classifies severity by nadir platelet counts 2
• Serious complications are frequent and may include:
  • Disseminated intravascular coagulation
  • Placental abruption
  • Acute renal failure
  • Pulmonary edema
  • Subcapsular liver hematoma or rupture
  • Retinal detachment
  • Cerebral hemorrhage

Pitfalls and Caveats

• In 20% of HELLP cases, there is no hypertension, and 5-15% present with minimal or no proteinuria 3
• Early recognition of hemolysis is most sensitively managed by determining serum haptoglobin 3
• AST and ALT elevations often precede the decrease in platelets 3
• Avoid high-dose corticosteroid treatment and repeated doses due to potential adverse effects on fetal brain development 1
• The risk of recurrence of hypertensive disease in subsequent pregnancies ranges from 27% to 48% 3

Referral Considerations

• Patients with complications such as pulmonary edema, acute renal failure, liver rupture, or extreme prematurity should be referred to a tertiary care center with maternal and neonatal intensive care facilities 4
• A multidisciplinary approach with prompt transfer to obstetric centers with expertise in this field is required for improved maternal-fetal outcomes 5