Abstract
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disorder characterised by an acquired mutation in haematopoietic stem cells leading to intravascular haemolysis and thrombotic events. In untreated women, pregnancy amplifies the risks of these manifestations, leading to increased maternal and neonatal morbidity and mortality. In the UK, eculizumab is the only approved treatment for PNH in pregnancy. Eculizumab was found to be safe in pregnancy and reduces PNH-related complications. Multidisciplinary team care between haematologists specialising in PNH, obstetricians, anaesthetists and the midwifery team remains crucial. This report describes a case of a woman with known PNH successfully treated with eculizumab in her first pregnancy. She had a vaginal delivery and suffered no complications associated with PNH. The woman and her child are well one year post-delivery. We discuss the pre-conception, antenatal, intrapartum and postnatal management steps to provide optimal care to a woman with PNH in pregnancy.
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