Successful Desensitization of a Patient with Possible IgE-Mediated Anaphylactic Reaction to FVIII/VWF Concentrate

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dc.authorideren, tuba/0000-0001-5471-0663
dc.authoridCelik, Velat/0000-0002-8893-9420
dc.authoridBeken, Burcin/0000-0001-7677-7690;
dc.authorwosideren, tuba/JTU-4270-2023
dc.authorwosidYazicioglu, Mehtap/AAB-9587-2022
dc.authorwosidCelik, Velat/IUN-0044-2023
dc.authorwosidBeken, Burcin/GPK-8389-2022
dc.authorwosidçelik, velat/AAD-7191-2020
dc.contributor.authorBeken, Burcin
dc.contributor.authorCelik, Velat
dc.contributor.authorOzdemir, Pinar Gokmirza
dc.contributor.authorEren, Tuba
dc.contributor.authorYazicioglu, Mehtap
dc.date.accessioned2024-06-12T10:51:37Z
dc.date.available2024-06-12T10:51:37Z
dc.date.issued2019
dc.departmentTrakya Üniversitesien_US
dc.description.abstractBackground: Type 3 von Willebrand disease (VWD) is a severe bleeding disorder with a prevalence of 1:1 million live births. There are several von Willebrand factor (VWF) replacement products used in the treatment of acute bleeding episodes or as prophylaxis. Patients who receive multiple transfusions have an increased risk of developing antibodies against these products. These antibodies can lead to life-threatening anaphylactic reactions. Previous studies have identified immune complex formation and complement activation as the trigger for anaphylaxis, rather than IgE. IgE-mediated anaphylaxis against VWF concentrates has not yet been published. Case: A 4-year-old female patient with type 3 VWD was referred to our hospital because of an anaphylactic reaction during FVIII/VWF concentrate (Immunate (R)) infusion. She had previously received FVIII/VWF concentrate (Haemate P (R)) infusions 8 times without any complications. She did not have antibodies against VWF and FVIII, and serum IgA level was normal. Since she needed factor replacement therapy as a result of a growing hematoma on her scalp, we performed skin prick and intradermal tests 2 days after the reaction. The prick test, with FVIII/VWF (Immunate), was negative, but the intradermal test was positive. We administered a 12-step desensitization protocol with FVIII/VWF concentrate (Immunate) successfully without any reactions. Conclusion: Anaphylactic reaction to factor replacement products is a major problem for patients with VWD, especially type 3 VWD requiring multiple factor infusions. We achieved a successful desensitization with FVIII/VWF concentrate in a patient who had an anaphylactic reaction during the infusion of this product. Our patient is important since she represents the first case of IgE-mediated anaphylaxis against VWF concentrate reported in the literature.en_US
dc.identifier.doi10.1089/ped.2018.0969
dc.identifier.endpage84en_US
dc.identifier.issn2151-321X
dc.identifier.issn2151-3228
dc.identifier.issue2en_US
dc.identifier.pmid31508262en_US
dc.identifier.scopus2-s2.0-85067499467en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage81en_US
dc.identifier.urihttps://doi.org/10.1089/ped.2018.0969
dc.identifier.urihttps://hdl.handle.net/20.500.14551/18418
dc.identifier.volume32en_US
dc.identifier.wosWOS:000471893700009en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherMary Ann Liebert, Incen_US
dc.relation.ispartofPediatric Allergy Immunology And Pulmonologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAnaphylaxisen_US
dc.subjectDesensitizationen_US
dc.subjectIge-Mediated Mechanismen_US
dc.subjectVon Willebrand Diseaseen_US
dc.subjectVon Willebrand Factor Concentrateen_US
dc.subjectVon-Willebrand-Diseaseen_US
dc.subjectFactor-Viiien_US
dc.subjectAlloantibodiesen_US
dc.subjectAntibodiesen_US
dc.subjectDiagnosisen_US
dc.subjectComplementen_US
dc.titleSuccessful Desensitization of a Patient with Possible IgE-Mediated Anaphylactic Reaction to FVIII/VWF Concentrateen_US
dc.typeArticleen_US

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