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Hla-Drb1*01:01, But Not Hla-Drb1:1503 or Hla-Drb1*11, Is Associated With Decreased Inhibitor Risk in Iranian Hemophilia a Patients Publisher Pubmed



Hosseini S1 ; Arabi S2 ; Yari F3 ; Pourfatollah A3 ; Rezaie N4 ; Moazezi S5 ; Aghaie A3
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Authors Affiliations
  1. 1. Biotechnology, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
  2. 2. Immunology, Department of Immunology, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran
  3. 3. Immunology, Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
  4. 4. Epidemiology, Non-Communicable Diseases Research Center, Endocrinology and Metabolism Research Institute, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Iranian Comprehensive Hemophilia Care Center (ICHCC), Iran

Source: Transfusion and Apheresis Science Published:2019


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. © 2019 Elsevier Ltd
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