FIORE, Mathieu; D'OIRON, Roseline; PILLOIS, Xavier; ALESSI, Marie-Christine

doi:10.1111/bjh.15087

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FIORE, Mathieu

D'OIRON, Roseline

PILLOIS, Xavier
Biologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases

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Article de revue

Ce document a été publié dans

British Journal of Haematology. 2018-04, vol. 181, n° 2, p. 173–182

Résumé en anglais

Glanzmann thrombasthenia (GT) is caused by inherited defects of the αIIb β3 platelet glycoprotein. This bleeding disorder can be treated with platelet transfusion therapy, but some patients will be immunized and begin to form anti-human leucocyte antigen (HLA) and/or anti-αIIb β3 antibodies. These antibodies can bind and interfere with the function of the transfused platelets, rendering treatment ineffective. However, platelet transfusion refractoriness attributable to HLA antibodies may be managed by the selection of compatible donors, although they are not always readily available, particularly in an emergency. Thus, anti-αIIb β3 antibodies represent one of the most severe complications in GT. Both genetic and environmental factors may contribute to the risk of anti-αIIb β3 development, but the underlying pathogenic mechanisms are still unknown. This review will summarize the current knowledge of the risk factors for development of anti-αIIb β3 antibodies in patients with GT and discuss how these findings may influence the clinical management of patients.< Réduire

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Mots clés en anglais

Autoantibodies

Glanzmann thrombasthenia

Humans

immunization

Immunization

platelet antibody

Platelet Glycoprotein GPIIb-IIIa Complex

Platelet Transfusion

rFVIIa

Risk Factors

Thrombasthenia

Transfusion Reaction

αIIbβ3