European Management of Glanzmann's Thrombasthenia: A Survey of Current Clinical Practice

Résumé fourni par la source

INTRODUCTION: Glanzmann's thrombasthenia is a rare inherited platelet disorder characterized by a lack of platelet aggregation. Patients tend to be diagnosed in early childhood with treatment strategies involving a multifaceted approach to prevent and manage bleeding episodes. Unfortunately, there is currently no European consensus regarding the management of GT. AIM: This initiative aimed to gain an understanding of current clinical management of GT across Europe, with the aim of aligning best practice and improving patient outcomes. METHODS: The authors, on behalf of the EAHAD Glanzmann Working Group, administered an online survey of 57 questions to European haematologists currently involved in the management of patients with GT. The survey covered topics related to diagnosis, treatment access and selection, immunization, peri-operative management and use of second-line therapies. RESULTS: Responses reflected physician consensus around some topics, including peri-operative treatment, use of recombinant factor VIIa, and concerns around antibody development. However, more varied responses were received on topics such as antibody screening (anti-αIIbβ3 antibodies screening conducted by ≤53% of respondents in all countries of interest except France), access to HLA-matched platelet concentrates (none or limited for 55% of respondents) and duration of platelet transfusions for major surgery (13%-31% for 1, 2, 3 and 4 or more days of transfusions). CONCLUSION: Establishing comprehensive guidelines to manage GT will enhance patient outcomes by ensuring patients receive high-quality and effective care as well as standardize care across different healthcare settings.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.