What are the diagnosis and treatment of HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Diagnosis and Treatment of HELLP Syndrome

Diagnosis

HELLP syndrome is diagnosed when a pregnant patient presents with hemolysis (confirmed by peripheral blood smear showing microangiopathic changes), elevated liver enzymes (AST/ALT), and platelet count below 100,000/mm³, typically in the setting of severe pre-eclampsia with blood pressure exceeding 160/90 mmHg. 1

Clinical Presentation

• Epigastric or right upper quadrant pain is the hallmark symptom, often accompanied by nausea, vomiting, and upper abdominal tenderness 2, 1
• Hypertension (>160/90 mmHg), proteinuria (>5g/24h), and peripheral edema develop rapidly 2, 1
• Critical pitfall: Symptoms frequently mimic acute cholecystitis, drug reactions, or idiopathic thrombocytopenia, leading to delayed diagnosis 2, 3

Essential Laboratory Investigations

Obtain these tests immediately upon suspicion:

• Complete blood count with peripheral smear to confirm microangiopathic hemolytic anemia 2, 4
• Platelet count (degree of thrombocytopenia correlates with liver dysfunction severity) 2, 1
• Liver function tests: AST, ALT, LDH (LDH reflects both hemolysis extent and hepatic dysfunction) 2, 4, 5
• Coagulation profile: PT, aPTT, fibrinogen, fibrin degradation products 2, 3
• Renal function: creatinine, urea, uric acid 2, 1
• Serum haptoglobin (low) and indirect bilirubin (elevated) support hemolysis 4, 6

Additional Diagnostic Studies

• Chest X-ray to exclude pulmonary edema 2, 1
• ECG for cardiac assessment 2, 1
• Abdominal ultrasound if hepatic hematoma or rupture is suspected (watch for right shoulder pain) 3

Treatment Algorithm

Step 1: Immediate Stabilization and Monitoring

The definitive treatment is prompt delivery, but stabilization must occur first to reduce maternal mortality (historically 3.4% but can reach 24%). 2, 1

• Insert central venous catheter in critically ill patients for fluid management (many patients are relatively hypovolemic) 2, 1
• Insert urinary catheter for hourly output monitoring 2, 1
• Initiate IV magnesium sulfate for seizure prophylaxis and neuroprotection 1, 3
• Control severe hypertension with IV hydralazine or labetalol to prevent intracranial hemorrhage 2, 1, 3
• Transfer to high-dependency unit or ICU for continuous monitoring of blood pressure, CVP, oxygen saturation, ECG, and urine output 2, 3

Step 2: Blood Product Replacement

• Transfuse platelets if count <50,000/mm³, especially before cesarean section 2, 1, 3
• Transfuse whole blood or packed red cells if hemoglobin <10 g/dL 2, 1, 3
• Administer fresh frozen plasma to correct hypovolemia and coagulopathy 2

Step 3: Timing and Mode of Delivery

Proceed to immediate delivery if any of the following are present:

• Worsening pre-eclampsia (increasing blood pressure, cerebral symptoms) 2, 1
• Deteriorating hepatic or renal function 2, 1
• Severe thrombocytopenia (especially <20,000/mm³, which increases hepatic hemorrhage risk) 1, 3
• Gestational age ≥32-34 weeks 2, 1
• Evidence of fetal distress or fetal maturity 2, 1

Cesarean section is required in 61.5-76% of cases due to obstetric indications 2, 1

Step 4: Anesthetic Management

• General anesthesia with propofol (minimal hepatic/renal metabolism) is preferred over neuraxial techniques given severe thrombocytopenia 1
• Rapid sequence induction with suxamethonium for airway control 2, 1
• Monitor blood glucose intraoperatively (severe hypoglycemia can occur) 2, 3

Step 5: Postpartum Management

The HELLP syndrome intensity peaks 24 hours after delivery, requiring continued intensive monitoring. 1, 4

• Continue magnesium sulfate for 24 hours postpartum 3
• Maintain strict fluid balance to avoid pulmonary edema—avoid aggressive fluid resuscitation 3
• Serial laboratory monitoring: CBC, platelets, liver enzymes, coagulation profile every 6-12 hours 3
• Monitor for complications: hepatic hemorrhage/hematoma, renal failure, pulmonary edema, DIC 1, 3
• LDH and platelet count are the two best tests to monitor disease course 4, 5

Critical Caveats

• Do NOT give corticosteroids to improve maternal outcomes in HELLP syndrome—while some studies show temporary hematologic improvement, current guidelines advise against their use for maternal benefit 3, 7
• Avoid aggressive fluid resuscitation that would be reflexive in other critically ill patients, as this significantly increases pulmonary edema risk 3
• Watch for delayed diagnosis: up to 30% of cases lack classic pre-eclampsia features initially 4, 6
• Platelet count <20,000/mm³ markedly increases hepatic hemorrhage risk and requires immediate intervention 3

Long-term Counseling

• Counsel regarding 19-27% recurrence risk in future pregnancies 3
• Consider aspirin prophylaxis (starting before 16 weeks gestation) in subsequent pregnancies 3