Anti-αIIb β3 immunization in Glanzmann thrombasthenia: review of literature and treatment recommendations
Language
EN
Article de revue
This item was published in
British Journal of Haematology. 2018-04, vol. 181, n° 2, p. 173–182
English Abstract
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 ...Read more >
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.Read less <
Keywords
Article RECHERCHE
English Keywords
Autoantibodies
Glanzmann thrombasthenia
Humans
immunization
Immunization
platelet antibody
Platelet Glycoprotein GPIIb-IIIa Complex
Platelet Transfusion
rFVIIa
Risk Factors
Thrombasthenia
Transfusion Reaction
αIIbβ3