Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience
Turk J Med Sci
; 51(4): 2135-2141, 2021 08 30.
Article
em En
| MEDLINE
| ID: mdl-33957723
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 andmethods:
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Trombastenia
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Complexo Glicoproteico GPIIb-IIIa de Plaquetas
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Plasma Rico em Plaquetas
Tipo de estudo:
Clinical_trials
/
Diagnostic_studies
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Observational_studies
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Prognostic_studies
/
Qualitative_research
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Humans
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Male
País/Região como assunto:
Asia
Idioma:
En
Revista:
Turk J Med Sci
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Turquia