Inhibitor-immunology-study. Evaluation of inhibitor development in haemophilia B.
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Inhibitor-immunology-study. Evaluation of inhibitor development in haemophilia B. / Wieland, I; Wermes, C; Eifrig, Barbara; Holstein, Katharina; Pollmann, H; Siegmund, B; Eberl, W; Kemkes-Matthes, B; Bidlingmaier, C; Kurnik, K; Lischetzki, G; Nimtz-Talaska, A; Eisert, R; Bogdanova, N; Doerk, T; Sykora, K-W.
In: HAMOSTASEOLOGIE, Vol. 31 Suppl 1, 2011, p. 57-60.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Inhibitor-immunology-study. Evaluation of inhibitor development in haemophilia B.
AU - Wieland, I
AU - Wermes, C
AU - Eifrig, Barbara
AU - Holstein, Katharina
AU - Pollmann, H
AU - Siegmund, B
AU - Eberl, W
AU - Kemkes-Matthes, B
AU - Bidlingmaier, C
AU - Kurnik, K
AU - Lischetzki, G
AU - Nimtz-Talaska, A
AU - Eisert, R
AU - Bogdanova, N
AU - Doerk, T
AU - Sykora, K-W
PY - 2011
Y1 - 2011
N2 - The development of inhibitors in haemophilia B is one of the most important complications of replacement therapy, affecting mortality and morbidity. Inhibitor development is based on complex immunological factors, and to date, only little is known about its underlying mechanisms. Here, we present first results of the haemophilia B group of our Inhibitor-Immunology study. Patients, methods: So far we have analysed 15 patients with haemophilia B. Four of them developed a high titre inhibitor; the remaining 11 had no inhibitor. We evaluated 9 SNPs in 8 genes (CD40, CTLA-4 , IL-1?, IL-10, TLR2 , TLR4, TLR9, TNF-?). We compared the distribution of these alleles between inhibitor and non-inhibitor haemophilia B patients and between haemophilia B patients and a normal male control population. HLA typing was performed in all patients. Results, discussion: There appears to be a trend towards a skewed distribution of TLR 9, IL-10 and CTLA4 alleles in haemophilia B patients. Due to the limited number these differences are, however, not statistically significant. The t-test of all patients with inhibitor versus without inhibitor was significant for HLA-A*03 and DPB1*0401 and borderline for DRB1*0201.
AB - The development of inhibitors in haemophilia B is one of the most important complications of replacement therapy, affecting mortality and morbidity. Inhibitor development is based on complex immunological factors, and to date, only little is known about its underlying mechanisms. Here, we present first results of the haemophilia B group of our Inhibitor-Immunology study. Patients, methods: So far we have analysed 15 patients with haemophilia B. Four of them developed a high titre inhibitor; the remaining 11 had no inhibitor. We evaluated 9 SNPs in 8 genes (CD40, CTLA-4 , IL-1?, IL-10, TLR2 , TLR4, TLR9, TNF-?). We compared the distribution of these alleles between inhibitor and non-inhibitor haemophilia B patients and between haemophilia B patients and a normal male control population. HLA typing was performed in all patients. Results, discussion: There appears to be a trend towards a skewed distribution of TLR 9, IL-10 and CTLA4 alleles in haemophilia B patients. Due to the limited number these differences are, however, not statistically significant. The t-test of all patients with inhibitor versus without inhibitor was significant for HLA-A*03 and DPB1*0401 and borderline for DRB1*0201.
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Young Adult
KW - Child
KW - Child, Preschool
KW - Genetic Predisposition to Disease/genetics
KW - Blood Coagulation Factor Inhibitors/blood/genetics
KW - Genes, MHC Class II/genetics
KW - Hemophilia B/blood/genetics
KW - Polymorphism, Single Nucleotide/genetics
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Young Adult
KW - Child
KW - Child, Preschool
KW - Genetic Predisposition to Disease/genetics
KW - Blood Coagulation Factor Inhibitors/blood/genetics
KW - Genes, MHC Class II/genetics
KW - Hemophilia B/blood/genetics
KW - Polymorphism, Single Nucleotide/genetics
M3 - SCORING: Journal article
VL - 31 Suppl 1
SP - 57
EP - 60
JO - HAMOSTASEOLOGIE
JF - HAMOSTASEOLOGIE
SN - 0720-9355
ER -
