Recognition and management of platelet-refractory bleeding in patients with Glanzmann’s thrombasthenia and other severe platelet function disorders

Recognition and management of platelet-refractory bleeding in patients with Glanzmann’s thrombasthenia and other severe platelet function disorders

Patients with rare qualitative platelet disorders or platelet function disorders (PFDs) may present to the hospital physician with severe bleeding episodes or excessive surgical bleeding. Although standard treatment consists of platelet transfusions, repeated transfusions may result in the development of antiplatelet antibodies (APA) or clinical refractoriness, rendering further platelet therap...

Density of Platelet GPIIb-IIIa and Bleeding Severity in Iranian Patients with Glanzmann’s Thrombasthenia

density of platelet gpiib-iiia and bleeding severity in iranian patients with glanzmann’s thrombasthenia

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Platelet function in a patient with thrombasthenia.

PLASMA CLOTTING FACTOR is deficient in most of the “experiments of nature” that have helped to further our understanding of the mechanism of blood coagulation and hemostasis, but in thrombasthenia the defect lies in the platelets which are unable to promote clot retraction. Thrombasthenic patients usually have a mild to moderately severe hemorrhagic diathesis, with a prolonged bleeding time. Th...

Control of severe bleeding episode in case of Glanzmann's thrombasthenia refractory to platelet transfusion therapy by administering recombinant factor VIIa.

Glanzmann's thrombasthenia is an autosomal recessive inherited platelet function defect. Though, quantitatively normal, the aggregation ability of platelets is reduced leading to bleeding episodes requiring transfusion of platelet concentrates. We describe a case of 13-year-old girl who had recurrent episodes of epistaxis since birth and was managed with multiple platelet concentrate transfusio...