Afficher la notice abrégée

Immunization against α β and α β in Glanzmann thrombasthenia patients carrying the French Gypsy mutation.

dc.rights.licenseauthentificationen_US
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorFIORE, Mathieu
dc.contributor.authorBAYAT, Behnaz
dc.contributor.authorPHUANGTHAM, Roongaroon
dc.contributor.authorBLOUIN, Laura
dc.contributor.authorHUGUENIN, Yoann
dc.contributor.authorBEIN, Gregor
dc.contributor.authorSANTOSO, Sentot
dc.date.accessioned2021-02-02T16:23:41Z
dc.date.available2021-02-02T16:23:41Z
dc.date.issued2021-01-01
dc.identifier.issn1538-7836en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/26108
dc.description.abstractEnEssentials 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.isoENen_US
dc.subject.enGP IIb/IIIa
dc.subject.enendothelial cells
dc.subject.eninherited/acquired platelet disorders
dc.subject.enintegrins
dc.subject.entransfusion medicine
dc.title.enImmunization against α β and α β in Glanzmann thrombasthenia patients carrying the French Gypsy mutation.
dc.title.alternativeJ Thromb Haemosten_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1111/jth.15117en_US
dc.subject.halSciences du Vivant [q-bio]/Médecine humaine et pathologie
dc.subject.halSciences du Vivant [q-bio]/Médecine humaine et pathologieen_US
dc.identifier.pubmed33090654en_US
bordeaux.journalJournal of Thrombosis and Haemostasisen_US
bordeaux.page255-261en_US
bordeaux.volume19en_US
bordeaux.hal.laboratoriesBiologie des maladies cardiovasculaires - U1034en_US
bordeaux.issue1en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.import.sourcepubmed
hal.identifierhal-03194572
hal.version1
hal.date.transferred2021-04-09T13:58:42Z
hal.exporttrue
workflow.import.sourcepubmed
bordeaux.COinSctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Journal%20of%20Thrombosis%20and%20Haemostasis&rft.date=2021-01-01&rft.volume=19&rft.issue=1&rft.spage=255-261&rft.epage=255-261&rft.eissn=1538-7836&rft.issn=1538-7836&rft.au=FIORE,%20Mathieu&BAYAT,%20Behnaz&PHUANGTHAM,%20Roongaroon&BLOUIN,%20Laura&HUGUENIN,%20Yoann&rft.genre=article


Fichier(s) constituant ce document

FichiersTailleFormatVue

Il n'y a pas de fichiers associés à ce document.

Ce document figure dans la(les) collection(s) suivante(s)

Afficher la notice abrégée