Obstetric and Gynaecological Challenges and Outcomes in Women and Girls With Glanzmann's Thrombasthenia.

introductionGlanzmann's thrombasthenia (GT) is an inherited platelet function disorder that may manifest with significant bleeding symptoms; in women and girls (W&Gs), heavy menstrual bleeding (HMB) is very common. GT in pregnancy is associated with an increased risk of postpartum haemorrhage (PPH).

aimThis study highlights the gynaecological and obstetric challenges experienced by W&Gs with GT, and reviews available treatment options.

methodsData regarding 38 W&Gs with GT were analysed from the ISTH REDCap registry, an international multi-centre database.

resultsAmong 38 W&Gs, 76% of Middle Eastern ethnicity, 100% reported HMB; 92% HMB since menarche, and 82% presented with acute HMB and were treated with platelets and packed red blood cells (pRBCs) transfusions in addition to hormonal therapies. Management of chronic HMB required a combination therapy including antifibrinolytics (tranexamic acid [TXA]), hormonal therapies, and recombinant factor VIIa (rFVIIa); rFVIIa was used in 50% of W&Gs. In 16 pregnancies, PPH was reported in 63% of deliveries, of which 83% required blood and platelet transfusions. Despite prophylactic haemostatic agents during labour and delivery in 8/9 pregnancies of women with known GT diagnosis, 78% experienced PPH. Thirty-one percent of neonates developed neonatal alloimmune thrombocytopenia (NAIT).

conclusionHMB and PPH are common bleeding complications in GT. Effective management of HMB and PPH in W&Gs with GT is challenging but can be achieved by a multidisciplinary team, often requiring a combination of haemostatic agents with hormonal therapies. Use of rFVIIa may limit the need for platelet transfusion, thus reducing alloimmunisation and the risk of developing NAIT.