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Anti-factor VIII an...
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Kahle, J.University Hospital Frankfurt
(author)
Anti-factor VIII antibodies in brothers with haemophilia A share similar characteristics
- Article/chapterEnglish2017
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LIBRIS-ID:oai:lup.lub.lu.se:da4c2432-4f11-4792-9e2b-b89165aadc90
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https://lup.lub.lu.se/record/da4c2432-4f11-4792-9e2b-b89165aadc90URI
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https://doi.org/10.1111/hae.13105DOI
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Language:English
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Summary in:English
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Introduction: The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is currently the most serious complication for patients with haemophilia A undergoing FVIII replacement therapy. Several genetic factors have been acknowledged as risk factors for inhibitor development. Aim: To analyze the influence of genetic factors on the nature of the humoral immune response to FVIII in eight brother pairs with inhibitors. Methods: The domain specificity of FVIII-specific IgG was analysed by antibody binding to FVIII fragments and homologue-scanning mutagenesis (HSM). The FVIII-specific IgG subclasses were measured by direct ELISA. Results: Of the 16 patient analysed with both methods, 12 had A2- and 13 had C2-specific IgG. The presence of A1-, A3- or C1-specific IgG was identified in nine of 14 patients analysed by HSM. IgG1, IgG2 and IgG4 subclasses contributed to the anti-FVIII IgG response, and the amount of FVIII-specific IgG1 (r = 0.66) and IgG4 (r = 0.69) correlated significantly with inhibitor titres. Patients with high concentrations of total anti-FVIII IgG (r = 0.69) or high inhibitor titres (r = 0.52) had a high proportion of FVIII-specific IgG4. Statistical analysis revealed trends/evidence that the subclass distribution (P = 0.0847) and domain specificity to HC/LC (P = 0.0883) and A2/C2 (P = 0.0011) of anti-FVIII IgG were more similar in brothers compared to unrelated subjects. Conclusion: Overall, our data provide a first hint that anti-FVIII IgG characteristics are comparable among haemophilic brothers with inhibitors. Whether genetic factors also influence the nature of patients' antibodies needs to be confirmed in a larger study population.
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Orlowski, A.University Hospital Frankfurt
(author)
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Stichel, D.University Hospital Frankfurt
(author)
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Healey, J. F.Children's Healthcare of Atlanta
(author)
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Parker, E. T.Children's Healthcare of Atlanta
(author)
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Donfield, S. M.Rho, Inc.
(author)
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Astermark, J.Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital(Swepub:lu)medf-jas
(author)
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Berntorp, E.Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital(Swepub:lu)medf-ebe
(author)
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Lollar, P.Children's Healthcare of Atlanta
(author)
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Schwabe, D.University Hospital Frankfurt
(author)
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Königs, C.University Hospital Frankfurt
(author)
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University Hospital FrankfurtChildren's Healthcare of Atlanta
(creator_code:org_t)
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In:Haemophilia: Wiley23:2, s. 292-2991351-8216
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Kahle, J.
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Orlowski, A.
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Stichel, D.
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Healey, J. F.
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Parker, E. T.
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Donfield, S. M.
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Astermark, J.
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Berntorp, E.
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Lollar, P.
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Schwabe, D.
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Königs, C.
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
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Haemophilia
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Lund University