Thrombocytopathy is a condition characterized by the presence of a normal number of platelets that are unable to adequately participate in hemostasis. The disease is manifested by mucocutaneous bleeding of the platelet type, a normal number of platelets, but an increased bleeding time and impaired functional properties of platelets (in particular, aggregation).


    • Drug-induced platelet dysfunction is the most common underlying cause of thrombocytopathy
    • Aspirin causes acetylation of platelet membranes, inhibiting the synthesis of prostaglandins necessary for the normal functioning of cells. With a large number of such defective platelets, the bleeding time is prolonged, hemorrhages are formed and bleeding in traumas increases. The effect of the product is preserved until the complete replacement of defective cells with a new population (traditionally within 3-7 days)
    • Other anti-inflammatory products (eg, indomethacin) cause similar dysfunction, but have a short duration of action that disappears when the product is discontinued.
    • Dysfunction in uremia. The mechanism of the pathology is unclear; uremic toxins are known to depolymerize high molecular weight factor VIII polymers. Genetic Aspects
    • Glanzmann-Negeli disease, type A (*273800, 17q21.1-q21.3, damage to the GP2B, GP3A, p or 90 genes. Clinically: hemorrhagic diathesis. Laboratory: normal clotting time and platelet count; insufficiency of thrombus retraction, impaired platelet morphology , decreased activity of glyceraldehyde phosphate dehydrogenase and pyruvate kinase in platelets, decreased likelihood of platelet adhesion, impaired platelet aggregation, anomalies of 11b / 1Pa glycoprotein.
    • Glanzmann-Negeli disease, type B (* 1734 70, 17q21.32, damage to the ITGB3, GP3A, R genes). Clinically: neonatal alloimmune thrombocytopenia, post-transfusion thrombocytopenia, hemorrhagic diathesis. Laboratory: insufficiency of platelet glycoprotein Sha, insufficiency of thrombus retraction, disorders of platelet aggregation

Treatment:. Bleeding in patients with uremia may decrease with hemodialysis. It is assumed that the restoration of high-molecular forms of factor VIII is able to temporarily correct the pathology; for this purpose, exogenous factor VIII is administered in the form of cryoprecipitate and the endogenous factor is activated by the administration of desmopressin. also von Willebrand’s disease, thrombocytopenia,

    • Trombok-

san A synthetase

    • a Deficiency of enzymes


    • D69.1 Qualitative platelet damage: thrombocytopathy, Glanzmann’s disease
    • D68.0 von Willebrand’s disease, angiohemophilia, factor VIII deficiency with vascular disorder, vascular hemophilia

MSH. Glanzmann-Negeli disease (273800,173470)

Literature. Bray PF: Inherited diseases of platelet glycoproteins:

considerations for rapid molecular characterization. Thromb. haemost.

72:492-502, 1994

Leave a Comment

Your email address will not be published. Required fields are marked *