TY  - JOUR
TI  - Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience
AB  - 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.Key words: Glanzmann thrombasthenia, glycoprotein IIb/IIIa, flow cytometry, platelet-rich plasma
AU  - Eser, Bülent
AU  - YILMAZ, SEMİH
AU  - Cetin, Aysun
AU  - ÇETİN, Mustafa
AU  - Koc, Basak
AU  - MUHTAROĞLU, Sabahattin
AU  - albayrak, canan
AU  - Albayrak, Davut
AU  - Sarper, Nazan
AU  - Bentli, Esma
AU  - Zulfikar, Bulent
AU  - zengin, emine
AU  - SARAYMEN, BERKAY
AU  - koker, Mustafa Yavuz
DO  - 10.3906/sag-2006-107
PY  - 2021
JO  - Turkish Journal of Medical Sciences
VL  - 51
IS  - 4
SN  - 1300-0144
SP  - 2135
EP  - 2141
DB  - TRDizin
UR  - http://search/yayin/detay/481008
ER  -