Glanzmann's thrombasthenia (GT) is a rare genetic bleeding disorder. The aim of our study was to evaluate the clinicopathological spectrum of this syndrome and to study the platelet glycoprotein IIb/IIIa complex and platelet antibodies by flow cytometry in a cohort of children with GT in a tertiary care center in Upper Egypt. Forty children with GT were assessed for the expression of GPIIb-IIIa on the platelet surface and platelet antibodies by using flow cytometry, to determine the most common GT subtypes among Egyptian children. By analysis of platelet GP IIb-IIIa by flow cytometry the classification of patients with GT in our study was type I GT (47.5%), type II GT (32.5%) and type III GT (20%). In this study, we have delineated that type I is the most common type of GT in Upper Egypt. Our data suggested that there is a good correlation between quantitative changes in the surface expression of platelet membrane glycoproteins detected by flow cytometry and the clinical severity of bleeding. Therefore, classifying of severity of bleeding in patients with GT could possibly aid the pediatricians and hematologists in the implementation of ideal prophylactic measures.
Keywords: Children; Flow cytometry; Glanzmann’s thrombasthenia; Platelet glycoprotein GPIIb-IIIa complex.