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- Title
Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience.
- Authors
SARAYMEN, Berkay; MUHTAROĞLU, Sabahattin; KÖKER, Mustafa Yavuz; SARPER, Nazan; ZENGİN, Emine; ALBAYRAK, Canan; ALBAYRAK, Davut; ZÜLFİKAR, Bülent; KOÇ ŞENOL, Başak; BENTLİ, Esma; YILMAZ, Semih; ÇETİN, Aysun; ESER, Bülent; ÇETİN, Mustafa
- Abstract
Background/aim: Glanzmann thrombasthenia (GT) is a rare autosomal recessively inherited bleeding disorder characterized by the quantitative (type 1 and type 2) or qualitative (type 3) deficiency in platelet membrane glycoprotein (GP) IIb/IIIa (CD41a/CD61) fibrinogen receptors. In type 1, 2, and 3, CD41a/CD61 expression is 5%, 5%–20% and above 20%, respectively. In this study, diagnosis of GT was confirmed and subgroups were identified in 32 Turkish patients by flow cytometry analysis. Materials and methods: CD41a/CD61 expression levels in platelet-rich plasma (PRP) obtained from peripheral venous EDTA blood samples were analyzed with a BD FACSCanto II flow cytometer (Becton Dickinson, Franklin Lakes, NJ, USA). GT subgroup analysis was performed by counting 50,000 events in the BD FACSDiva Software v6.1.3 program of the instrument. Results: In the present study, in blood samples of 32 patients from 23 families with GT and 22 healthy controls, co-expression levels of CD41a and CD61 in PRP was analyzed. 12 out of 23 families were consistent with type 1 GT (52.2%), 4 were consistent with type 2 GT (17.4%), and 7 were consistent with type 3 GT (30.4%). Conclusion: Especially due to consanguineous marriages, GT with various glycoprotein levels may be detected. As a result of the flow cytometry analysis of the present study with the highest GT patient population in Turkey, type 1 GT patients were the most common subgroup. In the determination of the GT subgroups; especially in the detection of type 3 GT, flow cytometry is the most sensitive glycoprotein analysis method. In addition to light transmission aggregometry, CD41a/CD61 study by flow cytometer confirms diagnosis when mutation analysis cannot be performed.
- Subjects
TURKEY; MEMBRANE glycoproteins; BLOOD coagulation factor XIII; DIAGNOSIS; PLATELET-rich plasma; BECTON-Dickinson (Company); SURFACE analysis; GLYCOPROTEIN analysis
- Publication
Turkish Journal of Medical Sciences, 2021, Vol 51, Issue 4, p2135
- ISSN
1300-0144
- Publication type
Article
- DOI
10.3906/sag-2006-107