HLA-DRB1*01:01, but not HLA-DRB1:1503 or HLA-DRB1*11, is associated with decreased inhibitor risk in Iranian hemophilia A patients. (October 2019)

• Record Type: Journal Article
• Authors: Hosseini, S.; Arabi, S.; Yari, F.; Pourfatollah, A.; Rezaie, N.; Moazezi, S.; Aghaie, A.
• Is Part Of: Transfusion and apheresis science. Volume 58:Number 5(2019)
• Page Start: 669
• Page End: 673
• Publication Date: 2019-10
• Subjects: Hemophilia -- Inhibitor -- HLA-DRB1*01:01 -- Coagulation factor VIII; Blood -- Transfusion -- Periodicals; Hemapheresis -- Periodicals; 615.39
• DOI: 10.1016/j.transci.2019.08.019
• Languages: English
• ISSNs: 1473-0502

Abstract

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.