title: Immunological study of platelet glycoproteins and alloantigens in families of patients with Glanzmann's thrombasthenia.
abstract: To explore the genetic transmission of Glanzmann's thrombasthenia (GT) we conducted a study of platelet glycoproteins and antigens using monoclonal and polyclonal antibodies and cytofluorometry. Twenty-one members of 4 different families in which at least one member was affected with GT were explored. The reactivity with AP2 (anti-GP IIb-IIIa complex, CD41a), AP3 (anti-GP IIIa) and antibodies against HPA-1a (PLA1) and HPA-3a (Leka) was lower than 5% in the platelets of 5 patients with GT type I. Nine obligatory carriers of the GT trait were tested by the same technique. The mean values of reactivity expressed as per cent of normal with AP2 (anti-CD41a) and AP3 (anti-GPIIIa) were 58.3 +/- 3.6% and 58.6 +/- 3.6% respectively, while the frequencies of HPA-1a (PLA1) and HPA-3a (Leka) negativity were 0/9 and 4/9 respectively. When both antigens were expressed they were detected at reduced levels. These results indicate that cytofluorometry is a simple technique for GT detection, but this method would nevertheless not appear to be sufficiently accurate to identify obligatory carriers in a general population.
authors.affiliation: INSERM U150, Institut des Vaisseaux et du Sang, Hôpital Lariboisière, Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Boizard-Boval
authors.forename: J L
authors.initials: JL
authors.lastname: Wautier
authors.forename: Y
authors.initials: Y
authors.lastname: Gruel
authors.forename: J P
authors.initials: JP
authors.lastname: Caen
mesh_terms: D000911:Antibodies, Monoclonal / Q000276:immunology; D016824:Antigens, Human Platelet / Q000235:genetics / Q000276:immunology*; D005260:Female; D005434:Flow Cytometry; D005455:Fluorescent Antibody Technique; D006580:Genetic Carrier Screening / Q000379:methods; D006801:Humans; D008297:Male; D010375:Pedigree; D010980:Platelet Membrane Glycoproteins / Q000235:genetics / Q000276:immunology*; D013915:Thrombasthenia / Q000235:genetics / Q000276:immunology*
chemical_list: D000911:Antibodies, Monoclonal; D016824:Antigens, Human Platelet; D010980:Platelet Membrane Glycoproteins