New Insights Into the Treatment of Glanzmann Thrombasthenia

Glanzmann thrombasthenia (GT) is a rare inherited autosomal recessive bleeding disorder of platelet function caused by a quantitative or qualitative defect of platelet membrane glycoprotein IIb/IIIa (integrin αIIbβ3), a fibrinogen receptor required for platelet aggregation. Bleeds in GT are variable and may be severe and unpredictable. Bleeding not responsive to local and adjunctive measures, as well as surgical procedures, is treated with platelets, recombinant activated factor VII (rFVIIa), or antifibrinolytics, alone or in combination.

Source: Transfusion Medicine Reviews - January 29, 2016 Category: Hematology Authors: Man-Chiu Poon, Giovanni Di Minno, Roseline d’Oiron, Rainer Zotz Source Type: research