Summary

• Considered to be a severe form of pre-eclampsia (sometimes called 'atypical pre-eclampsia') characterised by haemolysis (H), also expressed as microangiopathic haemolytic anaemia, elevated liver enzymes (EL), and low platelets (LP).
• Usually occurs antepartum between 27 and 37 weeks' gestation; 15% to 30% of cases present initially postpartum. Significant diagnostic and therapeutic challenge because only 80% to 85% of affected patients present typically with hypertension and proteinuria .
• Should be considered in any pregnant patient presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/upper abdominal pain until proven otherwise.
• Associated with progressive and sometimes rapid maternal and fetal deterioration.
• Although definitions and strict cut-off values for diagnosis are often arbitrary, adherence to widely accepted diagnostic criteria facilitates scientific reporting and communication.
• Early detection and aggressive management with a combination of IV magnesium sulphate, IV dexamethasone, control of blood pressure to prevent or minimise severe systolic hypertension, replacement of blood products as needed, and timely delivery of the fetus and placenta, appear to be the best and safest ways to arrest disease progression and reduce adverse outcomes. Maternal outcomes are improved considerably with this management; perinatal outcome depends predominantly upon the gestational age when delivery occurs.
• Although delivery is the only cure, serious manifestations of the disease continue into the immediate postpartum period.