Glanzmann's thrombasthenia in a twin pregnancy: A case report

Abstract

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive bleeding disorder caused by deficient or defective integrin αIIbβ3 on platelets, leading to impaired platelet aggregation. Pregnancy in women with GT poses significant challenges due to the increased risk of bleeding antenatally, intrapartum, and postpartum, as well as potential complications for the neonate, such as neonatal alloimmune thrombocytopenia (NAIT). This case report details the contemporary, multidisciplinary management of a 35-year-old primigravid woman with a dichorionic, diamniotic twin pregnancy and GT which included an elective caesarean section under general anaesthesia performed at 36 + 5 weeks of gestation, with recombinant factor VIIa (NovoSeven) and tranexamic acid (TXA) treatment preoperatively and pre-emptive uterotonic administration intraoperatively to minimise haemorrhage. Postpartum management included a seven-week course of TXA. This case highlights the importance of a proactive, multidisciplinary care plan to optimize pregnancy outcomes in women with GT, with a focus on minimizing the risk of severe haemorrhage.