HELLP Syndrome

HELLP syndrome is a severe form of preeclampsia with haemolysis, elevated liver enzymes and low platelets. It results from microangiopathy and liver injury. It is a serious complication of preeclampsia and carries high maternal and perinatal mortality.

• Some women have no hypertension or proteinuria at first.
• Onset is usually 28 to 36 weeks but some cases are postpartum.
• Misdiagnosed as gastritis, viral hepatitis or gallbladder disease.

Women have right upper quadrant or epigastric pain, nausea, vomiting, malaise and headache, with tenderness over the liver. Bleeding and jaundice may follow.

• Epigastric pain with vomiting in late pregnancy.
• Blood pressure may be only mildly raised.
• Differential diagnosis: acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, haemolytic uraemic syndrome, viral hepatitis, cholecystitis, gastritis, malaria, intrahepatic cholestasis, lupus flare, antiphospholipid syndrome.
• Complications: liver haematoma and rupture, disseminated intravascular coagulation, abruption, acute kidney injury, pulmonary oedema, eclampsia, stroke, postpartum haemorrhage, maternal and perinatal death.

The diagnosis needs all three laboratory features.

• Haemolysis: fragmented red cells on film, bilirubin 20 micromol/L or more, lactate dehydrogenase 600 IU/L or more.
• Elevated liver enzymes: aspartate aminotransferase 70 IU/L or more (or twice the upper limit).
• Low platelets: below 100 x 10^9/L.
• Creatinine, coagulation screen and fibrinogen, glucose, group and cross-match; liver ultrasound or CT for haematoma; cardiotocography and growth ultrasound.

The principle is stabilisation, magnesium sulphate, blood pressure control and delivery within 24 to 48 hours.

• Supportive: admit to a high dependency area; left lateral position; two cannulae; urinary catheter with hourly output; total fluids 80 mL per hour; avoid IM injections when platelets are below 50 x 10^9/L; avoid NSAIDs; monitor for liver pain and bleeding.
• Pharmacological: magnesium sulphate 4 g IV over 20 minutes then 1 g per hour for 24 hours after birth, stopping if reflexes are absent, respiratory rate is below 12 per minute or urine is below 30 mL per hour, and calcium gluconate 1 g IV over 10 minutes for toxicity; labetalol 20 mg IV over 2 minutes then 40 mg, 80 mg and 80 mg at 10 minute intervals (maximum 220 mg) or nifedipine immediate release 10 mg oral repeated once after 30 minutes or hydralazine 5 mg IV then 5 to 10 mg every 20 to 30 minutes (maximum 20 mg) for 160/110 mmHg or more; dexamethasone 10 mg IV 12-hourly for 2 doses before delivery below 34 weeks, for platelet recovery and fetal lung maturity, with betamethasone 12 mg IM twice, 24 hours apart, as the alternative for fetal lung maturity; oxytocin 10 IU IM after birth and no ergometrine; tranexamic acid 1 g IV over 10 minutes for bleeding.
• Procedures: delivery within 24 to 48 hours of diagnosis, at once for abruption, eclampsia, pulmonary oedema or fetal compromise; induction with misoprostol 25 micrograms vaginal 6-hourly (maximum 4 doses) or caesarean section; platelet transfusion of 1 adult dose for platelets below 20 x 10^9/L before vaginal birth and below 50 x 10^9/L before caesarean section; epidural only if platelets are 70 x 10^9/L or more; surgical packing or embolisation for liver haematoma.
• Special groups: kidney disease reduces magnesium sulphate to 0.5 g per hour with level monitoring; postpartum HELLP syndrome continues magnesium for 24 hours after diagnosis and needs steroids only if platelets are below 50 x 10^9/L.
• Red flags: severe upper abdominal pain, shoulder tip pain, platelets below 50 x 10^9/L, bleeding, seizure, oliguria, bilirubin rising, shock.
• Follow-up: blood tests every 6 to 12 hours until platelets rise and enzymes fall (usually 48 to 72 hours after birth); blood pressure at 6 hours, daily for 5 days, then at 2 and 6 weeks; review at 6 weeks and counsel on the risk of recurrence.