Quantitative Immunophemotyping of Platelet Surface Glycoproteins among Iranian Patients with Bernard-Soulier Syndrome

نویسندگان
A, Gharehbaghian ID 1
Gh, Toogeh ID 2
S, Hadjati ID 3
A, Farsinejad ID 4
M, Faranoush ID 5
N, Amirizadeh ID 6

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چکیده

Background: Bernard-Soulier syndrome is a rare inherited bleeding disease caused by quantitative or qualitative defect of GPIb/IX/V, a platelet complex that binds the Von Willebrand factor. The expression of GPIb-IX-V complex can be evaluated by flow cytometry and confirmed by the absence of ristocetin-induced platelet aggregation in platelet-rich plasma. The main aim ...

کلیدواژه‌ها
Bernard Soulier Syndrome, GPIb-IX-V, flow cytometry, bleeding.