Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience
| Autor: | Semih Yilmaz, Esma Bentli, Berkay Saraymen, Bulent Zulfikar, Başak Koç Şenol, Davut Albayrak, Canan Albayrak, Emine Zengin, Aysun Çetin, Bülent Eser, Nazan Sarper, Mustafa Cetin, Sabahattin Muhtaroglu, Mustafa Yavuz Köker |
|---|---|
| Přispěvatelé: | OMÜ, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Albayrak, Canan |
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Turkey glycoprotein IIb/IIIa Platelet Glycoprotein GPIIb-IIIa Complex Platelet Membrane Glycoproteins Fibrinogen Platelet membrane glycoprotein Gastroenterology Article Flow cytometry Young Adult Internal medicine Internal Medicine medicine Humans Platelet Child Glycoproteins medicine.diagnostic_test business.industry Platelet-Rich Plasma Glanzmann thrombasthenia flow cytometry Becton dickinson Integrin beta3 platelet-rich plasma General Medicine Platelet-rich plasma Case-Control Studies Child Preschool business Glycoprotein IIb/IIIa CD61 medicine.drug Thrombasthenia |
| Zdroj: | Turkish Journal of Medical Sciences |
| ISSN: | 1303-6165 |
| Popis: | SCI-Expanded Q4 WOS:000691664800006 PMID: 33957723 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|