glanzmann’s thrombasthenia pathogenesis, diagnosis, and current and emerging treatment options：血小板无力症的发病机制，诊断，和现有的和新兴的治疗选择.pdfVIP

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glanzmann’s thrombasthenia pathogenesis, diagnosis, and current and emerging treatment options：血小板无力症的发病机制，诊断，和现有的和新兴的治疗选择.pdf

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glanzmann’s thrombasthenia pathogenesis, diagnosis, and current and emerging treatment options：血小板无力症的发病机制，诊断，和现有的和新兴的治疗选择

Journal of Blood Medicine Dovepress open access to scientific and medical research Open Access Full Text Article R e v i e w Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options Tia Solh 1 Abstract: Glanzmann’s thrombasthenia (GT) is a genetic platelet surface receptor disorder Ashley Botsford2 of GPIIb/IIIa (ITG αIIbβ3), either qualitative or quantitative, which results in faulty platelet Melhem Solh3 aggregation and diminished clot retraction. Spontaneous mucocutaneous bleeding is common 1 and can lead to fatal bleeding episodes. Control and prevention of bleeding among patients with Department of Physician Assistant Studies, Philadelphia College of GT is imperative, and remains challenging. Local measures, including anti-fibrinolytic therapy, Osteopathic Medicine, Suwanee, with or without platelet transfusions, used to be the mainstay of therapy. However, in recent GA, USA; 2College of Health Care years the use of recombinant factor VIIa (rFVIIa) has increased significantly, with excellent Sciences, Nova Southeastern 3 response rates in treating and preventing hemorrhage among GT patients. Gene therapy and University, Orlando, FL, USA; The Blood and Marrow Transplant Group stem cell transplantation offer a potential cure of this disease, but both