Polymorphisms in the TNFA gene and the risk of inhibitor development in patients with hemophilia A.
(2006) In Blood 108(12). p.3739-3745- Abstract
- The HLA class I/II alleles and the tumor necrosis factor alpha (TNFA) locus are closely linked in the MHC complex. We have characterized the causative factor VIII mutation, HLA alleles as well as 4 polymorphisms (-827C > T, -308G > A, -238A > G, and 670A > G) in the TNFA gene in 164 patients (124 severe, 26 moderate, and 14 mild) in 78 families with hemophilia A enrolled in the Malmo International Brother Study (MIBS). Inhibitors were identified in 77.8% of patients with a single haplotype (Hap 2) and 72.7% of the patients with the TNFA -308 A/A genotype within this haplotype compared with 39.70/6 for TNFA -308 G/G patients and 46.9% for TNFA -308 G/A heterozygotes (OR 4.0; 95% CI, 1.4-11.5; P = .008). The association between... (More)
- The HLA class I/II alleles and the tumor necrosis factor alpha (TNFA) locus are closely linked in the MHC complex. We have characterized the causative factor VIII mutation, HLA alleles as well as 4 polymorphisms (-827C > T, -308G > A, -238A > G, and 670A > G) in the TNFA gene in 164 patients (124 severe, 26 moderate, and 14 mild) in 78 families with hemophilia A enrolled in the Malmo International Brother Study (MIBS). Inhibitors were identified in 77.8% of patients with a single haplotype (Hap 2) and 72.7% of the patients with the TNFA -308 A/A genotype within this haplotype compared with 39.70/6 for TNFA -308 G/G patients and 46.9% for TNFA -308 G/A heterozygotes (OR 4.0; 95% CI, 1.4-11.5; P = .008). The association between the -308 A/A genotype and inhibitors was enhanced in subgroups of patients with severe hemophilia (OR 19.2; 95% CI 2.4-156.5; P < .001) and with inversions (n = 75; OR, 11.8; 95% CI, 1.3-105.1; P = .013). Associations were found for the HLA A26 and B44 alleles, but these were not consistent in the subgroup analysis. Our data imply that the TNFA -308G > A polymorphism within Hap 2 is a useful marker and potential modulator of the immune response to replacement therapy in patients with hemophilia. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/159967
- author
- Astermark, Jan LU ; Oldenburg, Johannes ; Carlson, Joyce LU ; Pavlova, Anna ; Kavakli, Kaan ; Berntorp, Erik LU and Lefvert, Ann-Kari
- organization
- publishing date
- 2006
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Blood
- volume
- 108
- issue
- 12
- pages
- 3739 - 3745
- publisher
- American Society of Hematology
- external identifiers
-
- wos:000242309800025
- scopus:33845239946
- pmid:16926287
- ISSN
- 1528-0020
- DOI
- 10.1182/blood-2006-05-024711
- language
- English
- LU publication?
- yes
- id
- 073e42b4-0679-437c-b481-85afddf0433d (old id 159967)
- alternative location
- http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16926287&dopt=Abstract
- date added to LUP
- 2016-04-01 11:33:21
- date last changed
- 2022-04-12 22:02:09
@article{073e42b4-0679-437c-b481-85afddf0433d, abstract = {{The HLA class I/II alleles and the tumor necrosis factor alpha (TNFA) locus are closely linked in the MHC complex. We have characterized the causative factor VIII mutation, HLA alleles as well as 4 polymorphisms (-827C > T, -308G > A, -238A > G, and 670A > G) in the TNFA gene in 164 patients (124 severe, 26 moderate, and 14 mild) in 78 families with hemophilia A enrolled in the Malmo International Brother Study (MIBS). Inhibitors were identified in 77.8% of patients with a single haplotype (Hap 2) and 72.7% of the patients with the TNFA -308 A/A genotype within this haplotype compared with 39.70/6 for TNFA -308 G/G patients and 46.9% for TNFA -308 G/A heterozygotes (OR 4.0; 95% CI, 1.4-11.5; P = .008). The association between the -308 A/A genotype and inhibitors was enhanced in subgroups of patients with severe hemophilia (OR 19.2; 95% CI 2.4-156.5; P < .001) and with inversions (n = 75; OR, 11.8; 95% CI, 1.3-105.1; P = .013). Associations were found for the HLA A26 and B44 alleles, but these were not consistent in the subgroup analysis. Our data imply that the TNFA -308G > A polymorphism within Hap 2 is a useful marker and potential modulator of the immune response to replacement therapy in patients with hemophilia.}}, author = {{Astermark, Jan and Oldenburg, Johannes and Carlson, Joyce and Pavlova, Anna and Kavakli, Kaan and Berntorp, Erik and Lefvert, Ann-Kari}}, issn = {{1528-0020}}, language = {{eng}}, number = {{12}}, pages = {{3739--3745}}, publisher = {{American Society of Hematology}}, series = {{Blood}}, title = {{Polymorphisms in the TNFA gene and the risk of inhibitor development in patients with hemophilia A.}}, url = {{http://dx.doi.org/10.1182/blood-2006-05-024711}}, doi = {{10.1182/blood-2006-05-024711}}, volume = {{108}}, year = {{2006}}, }