The authors describe a method for the diagnosis of heredital platelet membranopathies by means of monoclonal antibodies against the main membrane glycoproteins of thrombocytes, glycoprotein Ib and IIIa. The platelets are differentiated in the flow fluorocytometer from other blood cells by the typical optic profile caused by their size and granular character. Monoclonal antibodies are bound to the appropriate membrane glycoprotein and their amount is then detected by means of a secondary antibody labelled with fluorescein. The intensity of fluorescence of individual platelets is proportional to the number of molecules of the appropriate glycoprotein on their surface. By the above technique a case of Glanzmann's thrombasthenia was diagnosed, a rare hereditary haemorrhagic disease, characterized by the absence or abnormal function of glycoprotein complex IIb/IIIa the platelet receptor for fibrinogen.

• MeSH
• glykoproteiny membrány trombocytů analýza   imunologie   MeSH
• lidé MeSH
• monoklonální protilátky MeSH
• předškolní dítě MeSH
• průtoková cytometrie * MeSH
• trombastenie diagnóza   genetika   MeSH
• Check Tag
• lidé MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• Názvy látek
• glykoproteiny membrány trombocytů MeSH
• monoklonální protilátky MeSH

The authors present a clinical description, detailed platelet function analysis, and certain biochemical parameters in two siblings with Glanzmann thrombasthenia (G. t.). The isolated occurrence of this disorder in the family corresponds with its autosomal recessive inheritance. In both cases blood platelets completely failed to aggregate. In contrast, the platelet interaction with ristocetin, reflecting their ability to adhere to the subendothelium, the so-called "shape change", the storage granule contents and their release and arachidonic acid metabolism were unaffected. Further, the aggregation abnormality was accompanied by marked procoagulant activity and clot retraction defects; these functions, similarly as aggregation, are implemented on the platelet surface. The analysis of blood platelet proteins, using two dimensional polyacrylamide electrophoresis, confirmed the absence of glycoprotein GP IIb and IIIa and a decrease of the fibrinogen content. The analysis of these findings in G. t. led to the contemporary concept that GP IIb and IIIa on the platelet surface act as receptors for platelet aggregation.

• MeSH
• agregace trombocytů MeSH
• buněčná membrána chemie   MeSH
• dospělí MeSH
• glykoproteiny membrány trombocytů analýza   MeSH
• lidé MeSH
• trombastenie krev   genetika   MeSH
• trombocyty chemie   ultrastruktura   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• glykoproteiny membrány trombocytů MeSH