What is the management plan for a patient diagnosed with Hellp (Hemolysis, Elevated Liver enzymes, and Low Platelet count) syndrome?

Management of HELLP Syndrome

The definitive treatment for HELLP syndrome is prompt delivery after initial stabilization, with immediate delivery indicated for worsening pre-eclampsia, severe thrombocytopenia, gestational age ≥32-34 weeks, or evidence of fetal distress. 1

Initial Stabilization and Diagnosis

HELLP syndrome represents the severe end of the pre-eclampsia spectrum and carries a maternal mortality of 3.4%, requiring aggressive early management. 2

Diagnostic Criteria

• Hemolysis: Presence of schistocytes on peripheral smear, total bilirubin >12 mg/L, or LDH >600 IU/L 3
• Elevated liver enzymes: Increased transaminases correlating with degree of thrombocytopenia 2, 1
• Low platelets: Platelet count <100,000/mm³ 3
• Severe pre-eclampsia markers: Blood pressure >160/90 mmHg, proteinuria >5g/24h, oliguria <400ml/24h, cerebral signs, or pulmonary edema 2, 1

Critical Initial Actions

• Blood pressure control: Initiate IV magnesium sulfate for seizure prophylaxis and neuroprotection 4
• Antihypertensive therapy: Use IV hydralazine or labetalol for severe hypertension (>160/110 mmHg) 4
• Comprehensive laboratory workup: Platelet count, white blood cell count, PCV, partial thromboplastin time, fibrinogen, fibrin degradation products, peripheral blood smear, liver function tests, creatinine, urea, and uric acid 2, 1
• Imaging: Chest X-ray to exclude pulmonary edema and ECG 2, 1
• Monitoring setup: Insert urinary catheter for hourly output monitoring; consider central venous catheter in critically ill patients 1, 4

Blood Product Management

Transfuse platelets if count is <50,000/mm³, particularly before cesarean section. 2, 1, 4

• Whole blood transfusion: Administer if hemoglobin <10 g/dL 2, 4
• Severe thrombocytopenia (<20×10⁹/L): Correlates with higher risk of hepatic hemorrhage and requires aggressive platelet support 4

Indications for Immediate Delivery

Delivery should proceed immediately when any of the following are present:

• Worsening pre-eclampsia (increasing blood pressure, cerebral symptoms, deteriorating hepatic or renal function) 2, 1
• Severe thrombocytopenia 2, 1
• Gestational age ≥32-34 weeks 2, 1
• Evidence of fetal distress 2, 1
• Evidence of fetal maturity 2, 1

Mode of Delivery

• Cesarean section rates are 61.5-76% due to obstetric indications, though vaginal delivery is acceptable if cervical conditions are optimal without maternal or fetal complications 2, 1, 3

Conservative Management Before 32 Weeks

• Expectant management may be considered before 32 weeks with intensive monitoring to allow corticosteroid administration for fetal lung maturity 5, 6, 3
• The Mississippi protocol includes IV dexamethasone, magnesium sulfate, antihypertensives, and intensive monitoring with arterial line 5
• Important caveat: The American College of Obstetricians and Gynecologists advises against corticosteroids to improve maternal outcomes in HELLP syndrome 4

Anesthetic Considerations

For general anesthesia, use drugs with minimal hepatic or renal metabolism.

• Induction: Propofol is recommended over thiopentone 1
• Rapid sequence intubation: Suxamethonium for airway management 2, 1
• Cardiovascular stability: Remifentanil provides excellent cardiovascular stability in high-risk patients 7
• Fentanyl: 5 µg/kg IV to attenuate hypertensive response to intubation 2
• Maintenance: 50% nitrous oxide with 0.5% isoflurane in oxygen 2

Post-Delivery Management

Transfer to high-dependency unit or ICU for 24 hours minimum with intensive monitoring. 2, 4

Monitoring Parameters

• Continuous blood pressure, oxygen saturation, ECG, and central venous pressure 2, 4
• Hourly urinary output to assess renal function 2, 4
• Serial complete blood counts to monitor platelets and hemoglobin 4
• Liver function tests to track enzyme levels 4
• Coagulation profile including fibrinogen and fibrin degradation products 4

Continued Medical Management

• Magnesium sulfate: Continue for 24 hours postpartum for seizure prophylaxis 4
• Blood pressure control: Oral labetalol, nifedipine, or methyldopa for non-severe hypertension; IV labetalol/hydralazine for severe hypertension 4
• Strict fluid balance: Avoid aggressive fluid resuscitation to prevent pulmonary edema 4

Monitoring for Complications

• Hepatic hemorrhage or hematoma: Watch for abdominal pain, epigastric pain, or right shoulder pain; perform abdominal ultrasound if suspected 4
• Hepatic failure: Early referral to transplant center if signs develop 4
• Hypoglycemia: Monitor glucose levels during management 1, 4
• Renal failure: If oliguria develops, measure urine sodium and osmolality to confirm diagnosis 2

Common Pitfalls

• Delayed diagnosis: Symptoms mimic acute cholecystitis, drug reactions, or idiopathic thrombocytopenia 2, 1, 4
• Fluid overload: Aggressive fluid resuscitation significantly increases pulmonary edema risk 4
• Inappropriate corticosteroid use: Do not give corticosteroids to improve maternal outcomes 4

Long-Term Counseling

• Recurrence risk: Counsel regarding increased risk in future pregnancies 4
• Aspirin prophylaxis: Consider for future pregnancies starting before 16 weeks' gestation 4

The condition typically resolves within days after delivery, with platelet counts normalizing by postpartum day 2 and liver enzymes returning to normal over the following week. 2