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Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference

Mathieu Fiore1,2, Janine-Sophie Giraudet3, Marie-Christine Alessi4,5

  • 1Laboratoire d'hématologie, Centre de Référence des Pathologies Plaquettaires, CHU de Bordeaux, Hôpital Cardiologique, Inserm U1034 - Biologie des Maladies Cardio-Vasculaires, Pessac, France. mathieu.fiore@chu-bordeaux.fr.

Orphanet Journal of Rare Diseases
|June 29, 2023
PubMed

Glanzmann thrombasthenia (GT) is a genetic bleeding disorder characterised by severely reduced/absent platelet aggregation in response to multiple physiological agonists. The severity of bleeding in GT varies markedly, as does the emergency situations and complications encountered in patients. A number of emergency situations may occur in the context of GT, including spontaneous or provoked bleeding, such as surgery or childbirth. While general management principles apply in each of these settings, specific considerations are essential for the management of GT to avoid escalating minor bleeding events. These recommendations have been developed from a literature review and consensus from experts of the French Network for Inherited Platelet Disorders, the French Society of Emergency Medicine, representatives of patients' associations, and Orphanet to aid decision making and optimise clinical care by non-GT expert health professionals who encounter emergency situations in patients with GT.

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