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

  • Majid Naderi Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical Sciences, Zahedan, Iran
  • Manijeh Habibpour Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
  • Shaban Alizadeh Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
  • Zahra Kashani Khatib Hematology Department, High Institute for Research and Education in Transfusion Medicine, Iranian Blood Transfusion Organization, Tehran, Iran
  • Akbar Dorgalaleh Department of Hematology, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran
  • Mohammed Awal Issah Department of Hematology, International Campus, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
  • Fatemeh Naadali Department of Hematology, School of Allied Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran
Keywords: Human platelet Ag-1, Human platelet Ag-5, Platelet therapy, Glanzmann thrombastenia

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. 

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Published
2018-01-05
How to Cite
1.
Naderi M, Habibpour M, Alizadeh S, Kashani Khatib Z, Dorgalaleh A, Awal Issah M, Naadali F. 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. ijhoscr. 12(1):43-8.
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Original Article(s)