What are the diagnostic criteria and treatment recommendations for HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelet count) in a pregnant patient after 20 weeks gestation?

HELLP Syndrome: Diagnostic Criteria and Management

HELLP syndrome requires three diagnostic components—hemolysis, elevated liver enzymes (AST/ALT), and platelets <100,000/mm³—and the only definitive treatment is prompt delivery after maternal stabilization, as this syndrome resolves only after delivery and carries a 3.4% maternal mortality with delayed intervention. 1, 2

Diagnostic Criteria

Core Laboratory Components (ACOG Criteria)

• Hemolysis must be confirmed by peripheral blood smear showing schistocytes (microangiopathic hemolytic anemia) resulting from endothelial damage with fibrin deposition 1
• Elevated liver enzymes: AST ≥70 U/L, with LDH >600 U/L serving as a dual marker reflecting both hemolysis extent and hepatic dysfunction 1, 3
• Thrombocytopenia: Platelet count <100,000/mm³ is the ACOG threshold for diagnosis and indicates severe thrombocytopenia with significant maternal risk 1, 2
• The degree of thrombocytopenia directly correlates with severity of liver dysfunction and predicts adverse maternal outcomes 1, 2

Clinical Context and Timing

• HELLP syndrome typically develops between 27-37 weeks gestation, with approximately 70% of cases occurring before delivery in the third trimester 2, 3
• Critical pitfall: 30% of cases occur or worsen postpartum within 48-72 hours after delivery, requiring continued intensive monitoring 1, 2
• Laboratory abnormalities peak 24 hours after delivery and may take up to 10 days to fully resolve 1
• HELLP syndrome can present without hypertension in 15% of cases and without proteinuria, so absence of these features does not rule out the diagnosis 2

Immediate Management Algorithm

Step 1: Maternal Stabilization (Before Delivery)

• Initiate magnesium sulfate immediately for seizure prophylaxis in all women with HELLP syndrome 2, 4
• Control severe hypertension (>160/90 mmHg) with IV labetalol or hydralazine, or oral labetalol/nifedipine for non-severe hypertension 2, 4
• Transfer to ICU or high-dependency unit for continuous monitoring of blood pressure, central venous pressure, urinary output, ECG, and oxygen saturation 2, 4
• Insert urinary catheter for hourly output monitoring (oliguria <400 mL/24h indicates severe disease) 2, 4

Step 2: Essential Pre-Delivery Workup

Before any surgical intervention, obtain: 1

• Platelet count, white blood cell count, PCV
• Partial thromboplastin time, fibrinogen, fibrin degradation products
• Peripheral blood smear
• Liver function tests (AST, ALT, LDH, bilirubin)
• Serum creatinine, urea, and uric acid
• Chest X-ray to exclude pulmonary edema
• ECG examination

Step 3: Rule Out Hepatic Complications

• 65% of symptomatic HELLP patients present with right upper quadrant or epigastric pain, which should immediately trigger abdominal imaging (ultrasound or CT) to rule out subcapsular hematoma or hepatic rupture 1, 2
• Fibrin deposition within hepatic sinusoids causes sinusoidal obstruction leading to hepatic ischemia, subcapsular hematomas, and potential hepatic rupture 2
• Right shoulder pain may indicate diaphragmatic irritation from hepatic bleeding 2

Step 4: Correct Coagulopathy Before Surgery

• Platelet transfusion is mandatory when platelets <50,000/mm³ before any surgical intervention 2, 4
• Strongly consider platelet transfusion at higher levels (up to <100,000/mm³) given increased risk of abnormal coagulation and bleeding complications 2
• Administer fresh frozen plasma to correct coagulopathy as needed 2, 4

Delivery Timing Decision Algorithm

Immediate Delivery Indications (≥34 Weeks or Any Gestational Age with Complications)

Deliver immediately after maternal stabilization if: 2

• Gestational age ≥34 weeks
• Maternal deterioration (worsening laboratory values, severe symptoms, hepatic complications) at any gestational age
• Fetal compromise (non-reassuring fetal status, intrauterine growth restriction with abnormal Doppler) at any gestational age
• Hepatic hemorrhage, infarct, or rupture identified on imaging
• Blood pressure exceeding 160/90 mmHg despite treatment 1
• Proteinuria >5 g/24 hours 1
• Oliguria <400 mL/24 hours 1
• Cerebral signs or symptoms 1
• Pulmonary edema 1

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
• Critical caveat: Delaying delivery waiting for laboratory values to normalize is dangerous—HELLP syndrome only resolves after delivery, and maternal mortality is 3.4% with delayed intervention 2

Mode of Delivery

• Cesarean section rate is typically >70% due to obstetric indications including unfavorable cervix, fetal distress, and maternal deterioration 2
• Regional anesthesia (epidural/spinal) is contraindicated with platelets <100,000/mm³ due to risk of epidural hematoma 2
• General anesthesia may be required if platelets preclude regional anesthesia 2

Post-Delivery Management (Critical 48-Hour Period)

Intensive Monitoring Requirements

• Continue magnesium sulfate for 24 hours post-delivery to prevent eclamptic seizures 4
• Maintain transfer to high-dependency unit or ICU with continuous blood pressure, ECG, oxygen saturation, and central venous pressure monitoring 4
• Serial laboratory monitoring every 6-12 hours for at least 24-48 hours postpartum: complete blood counts, liver function tests, coagulation profile, renal function tests 2, 4

Fluid Management

• Strict fluid balance is essential to avoid pulmonary edema, which should be continued for 24 hours after delivery 4
• Critical pitfall: Avoid aggressive fluid resuscitation that might be reflexively given in other critically ill patients, as this significantly increases pulmonary edema risk 4
• Administer fresh frozen plasma 400 mL cautiously to increase plasma volume, guided by CVP (target CVP 3-8 mmHg) 4

Blood Product Transfusion Thresholds

• Transfuse platelets if count <50,000/mm³ or if active bleeding is present 4
• Transfuse packed red blood cells if hemoglobin <10 g/dL to maintain oxygen-carrying capacity 4
• Administer fresh frozen plasma for coagulopathy correction 4

Complication Surveillance

• Monitor for hepatic hemorrhage or hematoma (perform abdominal ultrasound if symptoms develop, with early referral to transplant center if signs of hepatic failure develop) 4
• Monitor for acute renal failure/oliguria (<400 mL/24 hours) 4
• Monitor for pulmonary edema 4
• Platelets <20×10⁹/L correlate with higher risk of hepatic hemorrhage 4

What NOT to Do

• Do NOT administer corticosteroids to improve maternal HELLP outcomes—they are ineffective for maternal disease (though a single course may be given for fetal lung maturation between 24-34 weeks) 2, 4
• Do NOT delay delivery waiting for laboratory normalization 2
• Do NOT use regional anesthesia with platelets <100,000/mm³ 2
• Do NOT perform aggressive fluid resuscitation 4

Prevention and Future Pregnancy Counseling

• Daily aspirin 81 mg/day beginning at weeks 12-16 of gestation and continuing until delivery should be considered in patients with risk factors for preeclampsia (prior preeclampsia, multiple gestation, diabetes mellitus, hypertension, chronic kidney disease, or autoimmune disease) 5
• Counsel regarding 20% recurrence risk in future pregnancies 4
• Consider aspirin prophylaxis 150 mg nightly starting before 16 weeks gestation until 36 weeks or delivery in future pregnancies 4