Résumé
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. The use of flowcytometric analysis in diagnosis of Bernard-Souliar syndrome. 15 suspected BSS, 20 healthy persons as control group and 3 ITP patints were selected to be analysed for the presence of GPIb alpha and GPIIIa on the surface of platelets with the application of FITC conjugated monoclonal antibodies using flowcytometry. 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 GPIb? but a normal expression of GPIIIa on platelets. 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