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Review
. 2023 Jun 29;18(1):171.
doi: 10.1186/s13023-023-02787-2.

Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders

Mathieu Fiore  1   2 , Janine-Sophie Giraudet  3 , Marie-Christine Alessi  4   5 , Céline Falaise  6   5 , Dominique Desprez  7 , Roseline d'Oiron  8 , Sophie Voisin  9 , Marie-Françoise Hurtaud  10 , Hélène Boutroux  11 , Paul Saultier  12   5 , Cécile Lavenu-Bombled  13 , Gilles Bagou  14 , Xavier Dubucs  15 , Anthony Chauvin  16 , Christophe Leroy  17 , Francine Meckert  18 , François Kerbaul  18 , Nicolas Giraud  19 , Ambra Pühler  3 , Ana Rath  3
Affiliations
Review

Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders

Mathieu Fiore et al. Orphanet J Rare Dis. .

Abstract

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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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Screenshots of Orphanet website (A) and the French application of Orphanet for mobile phone (B) where emergency recommendations are easily accessible
See this image and copyright information in PMC

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