Immunization against α β and α β in Glanzmann thrombasthenia patients carrying the French Gypsy mutation.
dc.rights.license | authentification | en_US |
hal.structure.identifier | Biologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases | |
dc.contributor.author | FIORE, Mathieu | |
dc.contributor.author | BAYAT, Behnaz | |
dc.contributor.author | PHUANGTHAM, Roongaroon | |
dc.contributor.author | BLOUIN, Laura | |
dc.contributor.author | HUGUENIN, Yoann | |
dc.contributor.author | BEIN, Gregor | |
dc.contributor.author | SANTOSO, Sentot | |
dc.date.accessioned | 2021-02-02T16:23:41Z | |
dc.date.available | 2021-02-02T16:23:41Z | |
dc.date.issued | 2021-01-01 | |
dc.identifier.issn | 1538-7836 | en_US |
dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/26108 | |
dc.description.abstractEn | Essentials The c.1544+1G>A mutation was identified in Gypsy Glanzmann thrombasthenia (GT) patients. Gypsy GT patients express normal α β carrying HPA-1b epitopes. To demonstrate HPA-1a alloimmunization by modified antigen capture assays. Gypsy GT patients could develop anti-HPA-1a alloantibodies against β and α β . ABSTRACT: Background Glanzmann thrombasthenia (GT) is a rare bleeding disorder caused by the absence or the dysfunction of the platelet α β integrin. A founder mutation in the ITGA2B gene was previously identified in French Gypsy patients. Interestingly, this mutation was strongly linked to the human platelet antigen-1b (HPA-1b). The HPA-1bb Gypsy patients are at risk of isoimmunization against α β , as this complex is not expressed at their platelet surface. Tentatively, they would, however, not have an increased risk of developing anti-HPA-1a alloantibodies by exposure of α β on platelets from random platelet transfusions. However, the β chain can also associate with the α subunit expressed at the platelet surface. Because Gypsy GT patients express normal α β carrying HPA-1b epitopes, these patients might develop anti-HPA-1a alloantibodies reacting with α β and/or β . Objectives/Patients/Methods To demonstrate this hypothesis, sera from HPA-1bb (n = 5) and HPA-1ab (n = 1) Gypsy GT patients were investigated by modified antigen capture assay using platelets or stable transfected cells. Furthermore, stable transfected cells expressing either α β or α β3 together with soluble monomeric chimeric β (as absorbent) were used to differentiate anti-β and anti-α β reactivity. Results Only HPA-1bb patients developed alloantibodies reacting with HPA-1a cells. Further analysis showed that HPA-1bb patients developed anti-HPA-1a alloantibodies reacting with β and/or α β . Conclusion In this study, we found that HPA-1bb patients who failed to express α β on the platelet surface can develop alloantibodies against HPA-1a reacting with β as well as α β . This is of particular importance as anti-HPA-1a alloantibodies might cause fetal neonatal alloimmune thrombocytopenia and/or platelet transfusion refractoriness. | |
dc.language.iso | EN | en_US |
dc.subject.en | GP IIb/IIIa | |
dc.subject.en | endothelial cells | |
dc.subject.en | inherited/acquired platelet disorders | |
dc.subject.en | integrins | |
dc.subject.en | transfusion medicine | |
dc.title.en | Immunization against α β and α β in Glanzmann thrombasthenia patients carrying the French Gypsy mutation. | |
dc.title.alternative | J Thromb Haemost | en_US |
dc.type | Article de revue | en_US |
dc.identifier.doi | 10.1111/jth.15117 | en_US |
dc.subject.hal | Sciences du Vivant [q-bio]/Médecine humaine et pathologie | |
dc.subject.hal | Sciences du Vivant [q-bio]/Médecine humaine et pathologie | en_US |
dc.identifier.pubmed | 33090654 | en_US |
bordeaux.journal | Journal of Thrombosis and Haemostasis | en_US |
bordeaux.page | 255-261 | en_US |
bordeaux.volume | 19 | en_US |
bordeaux.hal.laboratories | Biologie des maladies cardiovasculaires - U1034 | en_US |
bordeaux.issue | 1 | en_US |
bordeaux.institution | Université de Bordeaux | en_US |
bordeaux.peerReviewed | oui | en_US |
bordeaux.inpress | non | en_US |
bordeaux.import.source | pubmed | |
hal.identifier | hal-03194572 | |
hal.version | 1 | |
hal.date.transferred | 2021-04-09T13:58:42Z | |
hal.export | true | |
workflow.import.source | pubmed | |
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