title: Inhibitor-immunology-study. Evaluation of inhibitor development in haemophilia B.
abstract: 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.
  
  
  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-1β, IL-10, TLR2 , TLR4, TLR9, TNF-α). 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.
authors: Pädiatrische Hämatologie & Onkologie, MHH Hannover. wieland.ivonne@mh-hannover.de
authors: I
authors: I
authors: Wieland
authors: C
authors: C
authors: Wermes
authors: B
authors: B
authors: Eifrig
authors: K
authors: K
authors: Holstein
authors: H
authors: H
authors: Pollmann
authors: B
authors: B
authors: Siegmund
authors: W
authors: W
authors: Eberl
authors: B
authors: B
authors: Kemkes-Matthes
authors: C
authors: C
authors: Bidlingmaier
authors: K
authors: K
authors: Kurnik
authors: G
authors: G
authors: Lischetzki
authors: A
authors: A
authors: Nimtz-Talaska
authors: R
authors: R
authors: Eisert
authors: N
authors: N
authors: Bogdanova
authors: T
authors: T
authors: Doerk
authors: K-W
authors: KW
authors: Sykora
mesh_terms: D000293:Adolescent; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood* / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005802:Genes, MHC Class II / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D002836:Hemophilia B / Q000097:blood* / Q000235:genetics*; D006801:Humans; D008297:Male; D020641:Polymorphism, Single Nucleotide / Q000235:genetics*; D055815:Young Adult
chemical_list: D019774:Blood Coagulation Factor Inhibitors