title: PMA induces platelet activation of specific antigens (CD62/CD63) in GpIIb-IIIa deficient platelets from Glanzmann's thrombasthenia.
abstract: Glanzmann's thrombasthenia (GT) is a hereditary platelet disorder resulting from a quantitative or qualitative deficiency of the major platelet membrane complex GPIIb-IIIa (CD41) required for platelet aggregation. We investigated by flow cytometry, the expression of CD41, fibrinogen, and of two platelet activation-related antigens, CD62 and CD63, (i) before and after activation of platelets by PMA, and (ii) on the surface and within the cytoplasm of resting platelets, after permeabilization by saponin. Platelets from a series of normal subjects and from nine members of two GT families, were reacted with FITC-conjugated antibodies and analyzed on a flow cytometer. Fluorescence intensities measured on normal and GT platelets were quantified by using calibrated beads. Results showed lack of both GPIIb-IIIa and fibrinogen, on the platelet surface and also within the cytoplasm in five of these GT patients, whereas GPIIb-IIIa and fibrinogen remained normal in the four other cases. However, CD62 and CD63 antigenic levels were found within normal range for all members of these families, after PMA stimulation and also after platelet permeabilization. This work therefore showed that the lack of CD41 in GT, which causes strong disturbance of platelet aggregation, may not be associated with the deficiency of activation pathways.
authors.affiliation: Laboratoire Central d'Hématologie, Hôpital de Cimiez, Nice, France.
authors.forename: F
authors.initials: F
authors.lastname: Baudouin-Brignole
authors.forename: J
authors.initials: J
authors.lastname: Bayle
authors.forename: A
authors.initials: A
authors.lastname: Goguel
authors.forename: P J
authors.initials: PJ
authors.lastname: Philip