HELLP syndrome is a group of symptoms that occurs in pregnant women who have pre-eclampsia or eclampsia and who also show signs of liver damage and abnormalities in blood clotting.
It is characterised by:

• Haemolysis
• EL (elevated liver) enzymes
• LP (low platelet) count

Epidemiology

HELLP syndrome occurs in 0.5 to 0.9% of all pregnancies and in 10-20% of cases with severe pre-eclampsia.¹

• It may occur well before term.²
• HELLP may occur after pre-eclampsia is diagnosed (the norm) or be the first warning of pre-eclampsia when misdiagnosed as, for example, hepatitis or thrombotic thrombocytopenic purpura (TTP).
• It may occur in association with antiphospholipid syndrome.³
• Smoking in pregnancy has been found to cause an 80% reduction in the risk of developing HELLP.⁴

Presentation

80% of women with HELLP present before term.

Symptoms

Progressive nausea and vomiting
Upper abdominal pain
Headache
Vision problems

Signs

Jaundice
Upper abdominal tenderness, especially in the right upper quadrant
Hepatomegaly
Easy bruising/purpura

Investigations

Liver function tests - AST, ALT and LDH elevated.
FBC - Haemoglobin and platelet count reduced.

Differential diagnosis⁵

• Acute fatty liver of pregnancy
• TTP⁶
• Haemolytic uraemic syndrome
• Acute exacerbation of systemic lupus erythematosus

Management

The main treatment is to deliver the baby as soon as possible, even if premature, since liver function in the mother gets worse very quickly. Problems with the liver can be harmful to both mother and child.

Pharmacological

• Dexamethasone - in gestational ages between 24 and 34 weeks most authors prefer a single course of corticosteroid therapy for fetal lung maturation, either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg of dexamethasone 12 hours apart before delivery. Standard corticosteroid treatment is of uncertain clinical value in the maternal HELLP syndrome. High-dose treatment and repeated doses should be avoided for fear of long-term adverse effects on the fetal brain.¹
• Plasma exchange therapy has been successfully used in selected patients with HELLP, who have organ failure or who are refractory to treatment.

Surgery

Women with severe liver damage may need liver transplantation.

Prognosis

• If HELLP is not treated early, up to 25% of women may develop serious complications.
• Without treatment there is a significant mortality.
• The mortality rate among babies born to mothers with HELLP syndrome varies and depends mainly on gestation and birth weight.

Complications

• The maternal liver may haemorrhage or rupture.⁷
• Permanent liver damage or necrosis,⁸ which may need transplantation, may occur if delivery is delayed.
• Intraventricular haemorrhage with subsequent hydrocephalus has been reported.⁹
• Retinal detachment and other eye problems have been reported.¹⁰
• Transient diabetes insipidus may follow HELLP syndrome.¹¹