Immunogenetic Determinants of Therapeutic Resistance in Glanzmann Thrombasthenia
DOI:
https://doi.org/10.48047/ymc6ny10Keywords:
Immunogenetic Determinants, Therapeutic Resistance, Glanzmann ThrombastheniaAbstract
Background: Glanzmann thrombasthenia (GT) is a rare inherited platelet function disorder characterized by qualitative or quantitative defects in the platelet glycoprotein IIb/IIIa (αIIbβ3) integrin, leading to impaired platelet aggregation and a lifelong bleeding tendency. Despite advances in supportive management, including platelet transfusions and recombinant activated factor VII (rFVIIa), a subset ofpatients demonstrates poor or diminishing therapeutic response
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References
George JN, Caen JP, Nurden AT. Glanzmann’s thrombasthenia: the spectrum of clinical disease. Blood. 1990;75(7):1383-1395.
Nurden AT, Fiore M, Nurden P, Pillois X. Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models. Blood. 2011;118(23):5996-6005.
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