A survey on platelet surface glycoprotiens in patients with Bernard-Souliar syndrome by flowcytometry

Abstract Background: The gold standard diagnosis of the Bernard-Souliar syndrome (BSS), a rare disease, is to prove the absence of Ib/IX surface complex on platelets with the use of aggregometric methods. Flowcytometry is an ideal method in analysis of surface markers on cells. Objective: The use of flowcytometric analysis in diagnosis of Bernard-Souliar syndrome. Methods: 15 suspected BSS, 20 healthy persons as control group and 3 ITP patints were selected to be analysed for the presence of GPIbα and GPIIIa on the surface of platelets with the application of FITC conjugated monoclonal antibodies using flowcytometry. Findings: All healthy persons in control group and 3 ITP patients showed normal expression of both glycoprotiens on platelets using flowcytometry. All 15 suspected BSS patients showed lack of GPIbα but a normal expression of GPIIIa on platelets. Conclusion: The application of flowcytometry for diagnosis of BSS is a quick, accurate, and precise method, which together with aggregometric method can be used for diagnosis of BSS.