Management of HELLP Syndrome

Diagnosis and Initial Assessment

HELLP syndrome is characterized by hemolysis, elevated liver enzymes, and low platelet count, typically occurring as a severe variant of pre-eclampsia 3
Clinical presentation includes epigastric pain, upper abdominal tenderness, proteinuria, hypertension, jaundice, nausea, and vomiting 3
Severe pre-eclampsia is diagnosed when arterial pressure exceeds 160/90 mmHg, proteinuria >5g/24h, oliguria <400ml/24h, cerebral signs, or pulmonary edema 4
The degree of thrombocytopenia correlates with the severity of liver dysfunction 4

Control blood pressure using IV magnesium sulfate or hydralazine 5, 6
Perform comprehensive laboratory investigations including platelet count, white blood cell count, PCV, partial thromboplastin time 7
Perform comprehensive laboratory investigations including fibrinogen concentration, fibrin degradation products, peripheral blood smear 7
Perform comprehensive laboratory investigations including liver function tests, creatinine, urea, and uric acid levels 8
Obtain chest X-ray to exclude pulmonary edema and ECG 8
Insert urinary catheter for hourly monitoring of output 9
Consider central venous catheter in critically ill patients for fluid management 9

Administer platelet transfusion if count is less than 50,000/mm³, especially if delivery by cesarean section is planned 9, 7
Transfuse whole blood if hemoglobin concentration is less than 10 g/dL 9

Indications for immediate delivery include worsening pre-eclampsia, deteriorating hepatic or renal function, severe thrombocytopenia, gestational age at or beyond 32-34 weeks, evidence of fetal distress, and evidence of fetal maturity 1, 6, 4, 5, 8
Mode of delivery: Cesarean section rates are high (61.5-76%) due to obstetric indications 1

If general anesthesia is required, use drugs with minimal hepatic or renal metabolism (propofol is recommended) 7
Consider rapid sequence induction with suxamethonium for airway management 5, 7

Continue blood pressure control and fluid management 5
Watch for complications including hemorrhage, renal failure, and pulmonary edema 3

Maternal mortality rate has been reported at 3.4% in some series, highlighting the severity of this condition 1
Delayed diagnosis is common as symptoms may mimic other conditions (acute cholecystitis, drug reactions, idiopathic thrombocytopenia) 3
Hypoglycemia can occur and should be monitored during management 6