[Variant of Paris-I Lariboisière thrombasthenia, a molecular anomaly of the IIb-IIIa platelet glycoprotein complex].
Platelet GP IIb-IIIa complex is missing or strongly reduced in thrombasthenia type I or II; in parallel the binding of fibrinogen is either nil or strongly reduced after platelet activation and these platelets do not aggregate. In the platelets of the described variant, GP IIb-IIIa level and PLA1 antigen are around 50% of the normal but the fibrinogen sites are either missing or unavailable. The hypothesis is that the patient's platelets lack the specific receptor site for fibrinogen at the GP IIb-IIIa level. However, platelet fibrinogen is normal as is the clot retraction. The study of this variant allows new concepts on platelet aggregation and clot retraction.
D001791:Blood Platelet Disorders / Q000097:blood*; D001792:Blood Platelets / Q000378:metabolism*; D003018:Clot Retraction; D005340:Fibrinogen / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism*; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism*; D008875:Middle Aged; D010974:Platelet Aggregation; D010980:Platelet Membrane Glycoproteins
D006023:Glycoproteins; D008565:Membrane Proteins; D010980:Platelet Membrane Glycoproteins; D005340:Fibrinogen
Variant de thrombasthénie Paris-I Lariboisière, anomalie moléculaire du complexe glycoprotéinique plaquettaire IIb-IIIa.