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Study of the Relationship Between Hpa-1 and Hpa-5 Gene Polymorphisms and Refractory to Platelet Therapy and Recombinant Factor Vii in Glanzmann Thrombasthenia Patients in Southeast of Iran



Naderi M1 ; Habibpour M2 ; Alizadeh S2 ; Khatib ZK3 ; Dorgalaleh A4 ; Issah MA5 ; Naadali F2
Authors
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Authors Affiliations
  1. 1. Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, Iran
  2. 2. Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Hematology Department, High Institute for Research and Education in Transfusion Medicine, Iranian Blood Transfusion Organization, Tehran, Iran
  4. 4. Department of Hematology, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran
  5. 5. Department of Hematology, International Campus, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran

Source: International Journal of Hematology-Oncology and Stem Cell Research Published:2018

Abstract

Background: Glanzmann Thrombasthenia (GT) is a rare autosomal disease. HPA (Human Platelet Alloantigen) is a surface polymorphic alloantigen of platelets. This study was intended to investigate and compare the polymorphism of HPA-1 and HPA-5 genes in two groups of GT patients, with and without resistance to platelet and recombinant factor VII therapy. Materials and Methods: This case control study was performed on GT patients (n=16) with resistance to platelet therapy and recombinant factor VII and control group of GT patients (n=16) without resistance to platelet therapy and recombinant factor VII. The consent form was completed by each patient. Gene polymorphisms of HPA-1 and HPA-5 were investigated using SSP-PCR, and the obtained data were analyzed using statistical software SPSS16.0. Results: The results indicated no significant relationship between the studied genes and their resistance to platelet therapy and recombinant factor VII. The frequencies of HPA-1 genotype a/a were 98% and 94% in patient and control groups, respectively. The frequency of allele b was found to be less than allele a. The value of this allele was 4% in patient group and 1% in control group. In addition, the HPA-5a/a (98%) was the most frequent alloantigen?? (check it) in both groups. Seven percent (7%) of the patients had the HPA-5a/b genotype, and the HPA-5b/b was found to be absent in these individuals. Conclusion: According to the results obtained, it could be concluded that these genes play no role in resistance to platelet therapy. © 2018, Tehran University of Medical Sciences (TUMS). All rights reserved.