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Anti-factor VIII an...
Anti-factor VIII antibodies in brothers with haemophilia A share similar characteristics
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- Kahle, J. (författare)
- University Hospital Frankfurt
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- Orlowski, A. (författare)
- University Hospital Frankfurt
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- Stichel, D. (författare)
- University Hospital Frankfurt
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- Healey, J. F. (författare)
- Children's Healthcare of Atlanta
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- Parker, E. T. (författare)
- Children's Healthcare of Atlanta
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- Donfield, S. M. (författare)
- Rho, Inc.
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- Astermark, J. (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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- Berntorp, E. (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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- Lollar, P. (författare)
- Children's Healthcare of Atlanta
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- Schwabe, D. (författare)
- University Hospital Frankfurt
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- Königs, C. (författare)
- University Hospital Frankfurt
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(creator_code:org_t)
- 2016-11-08
- 2017
- Engelska.
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 23:2, s. 292-299
- Relaterad länk:
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http://dx.doi.org/10...
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https://lup.lub.lu.s...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- Anti-factor VIII antibodies
- Epitope mapping
- Factor VIII inhibitors
- Haemophilia A
- IgG subclasses
Publikations- och innehållstyp
- art (ämneskategori)
- ref (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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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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visa fler...
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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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visa färre...
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- MEDICIN OCH HÄLSOVETENSKAP
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och Klinisk medicin
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och Hematologi
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Haemophilia
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Lunds universitet