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

Abstract

Introduction

Glanzmann'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).

Aim

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

Methods

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

Results

Among 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).

Conclusion

HMB 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.