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

Year: 2025

Type: article

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.

Source: Haemophilia

DOI: 10.1111/hae.70030

Authors: Deborah Obeng‐Tuudah, Ahmad Tarawah, Melike Ozkan, Rezan Abdul‐Kadir

Institutions: National Health Service, The Royal Free Hospital, University College London

Language: English

Topic: Platelet Disorders and Treatments

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,760