HELLP Syndrome

HELLP syndrome is a group of symptoms that occur 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:

• H aemolysis
• EL (elevated) liver enzymes
• LP (low platelet) count

HELLP syndrome occurs in approximately 3% of pregnant women with pre-eclampsia or eclampsia. Pre-eclampsia may be mild or severe, but HELLP syndrome is associated with more serious cases.

• It may occur well before term.¹
• HELLP may occur after pre-eclampsia is diagnosed ( the norm) or be the first warning of pre-eclampsia when misdiagnosed as e.g. hepatitis or thrombotic thrombocytopenic purpura.
• It may occur in association with Antiphospholipid syndrome.²
• Smoking in pregnancy has been found to affect an 80% reduction in the incidence of HELLP.³

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

Liver function tests may be elevated; AST, ALT and LDH.
FBC and platelet counts may be low.

• Acute fatty liver of pregnancy
• Thrombotic thrombocytopenic purpura⁵
• Haemolytic uraemic syndrome
• Acute exacerbation of systemic lupus erythematosus

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 - a Cochrane review (2004) looked at RCTs where this had replaced standard therapy.⁶Although there were no significant differences in the primary outcomes of maternal mortality and morbidity (due to placental abruption, pulmonary oedema and liver hematoma or rupture), with secondary maternal outcomes, there was a tendency to a greater platelet count increase over 48 hours, statistically significantly less mean number of hospital stay days and mean interval (hours) to delivery.
  There were no significant differences in perinatal mortality or morbidity due to respiratory distress syndrome, need for ventilatory support, intracerebral haemorrhage, necrotizing enterocolitis and a five minute Apgar less than seven. However, the mean birthweight was significantly greater in the group allocated to dexamethasone.
• 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.

If HELLP is not treated early up to 25% of women may develop serious complications.⁷Without 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.

The maternal liver may haemorrhage or rupture.⁸
Permanent liver damage or necrosis,⁹ which may need transplantation, may occur if delivery is delayed.
Intraventricular haemorrhage with subsequent hydrocephalus has been reported.¹⁰
Retinal detachment and other eye problems have been reported.¹¹
Transient Diabetes Insipidus may follow HELLP syndrome.¹²