1. HLA-DRB1*01:01, but not HLA-DRB1:1503 or HLA-DRB1*11, is associated with decreased inhibitor risk in Iranian hemophilia A patients
- Author
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S. Moazezi, S. Arabi, S. Hosseini, A A Pourfatollah, F. Yari, Afsaneh Aghaie, and N. Rezaie
- Subjects
Adult ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Population ,Iran ,030204 cardiovascular system & hematology ,Hemophilia A ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Gene Frequency ,Risk Factors ,hemic and lymphatic diseases ,Internal medicine ,Humans ,Medicine ,Allele ,Child ,education ,HLA-DRB1 ,Genotyping ,Allele frequency ,Alleles ,Aged ,education.field_of_study ,Factor VIII ,Blood Coagulation Factor Inhibitors ,business.industry ,Genetic disorder ,Infant ,Hematology ,Middle Aged ,medicine.disease ,Coagulation ,Child, Preschool ,Female ,Complication ,business ,HLA-DRB1 Chains ,030215 immunology - Abstract
Background/objective Hemophilia A is a genetic disorder through which patients suffer from recurrent bleeding. This can be caused by a defect in human plasma coagulation factor VIII. High incidence of FVIII inhibitors in some severe hemophilia A patients after FVIII therapy is a considerable complication. Determination of good predictive factors can improve the safety of this treatment. HLA-II have been shown as a predictive element for inhibitor development. The goal of this study is to determine the association between HLA-DRB1*15:03, HLA-DRB1*11 and HLA-DRB1*01:01 alleles and FVIII inhibitors in severe hemophilia A patients in Iran. Materials/methods HLA-DRB1 genotyping was performed using Multiplex sequences Specific Primers (PCR-SSP) in two groups of severe hemophilia A patients comprising 51 and 50 individuals with and without FVIII inhibitors respectively. The levels of inhibitor were determined through Nijmegen-modified Bethesda assay. HLA-DRB1 allele frequencies were compared between groups by using multiple logistic regression models. Results HLA-DRB1*01:01 allele frequency was significantly higher in patients without inhibitor ORadj: 2.7 (95%CI: 1.08, 6.97; P = 0.034). There wasn’t any statistically significant difference in HLA-DRB1*11 allele frequency between groups ORadj: 0.7 (95%CI: 0.27, 1.82; P = 0.47). There was no connection between HLA-DRB1*15:03 and inhibitor development ORadj: 0.94 (95%CI: 0.38, 2.35; P = 0.94). Conclusion An association between HLA-DRB1*01:01 and paucity of FVIII inhibitor showed that this allele has probably a protective effect in severe hemophilia A patients in Iran. Determination of the predictive and protective alleles are beneficial in pre-treatment activities and decrease the risk of unsuccessful therapy with FVIII in each population.
- Published
- 2019
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