Neşe YARALI, Abdurrahman KARA, Bahattin TUNÇ, İkbal OK BOZKAYA

Trombosit Refrakterliği Olan Glanzmann Trombastenili Hastada Travmatik Hifemanın Rekombinan Faktör VIIa ile Başarılı Tedavisi

Glanzmann trombastenisi (GT) yaşam boyu kanamaya yatkınlıkla karakterize olan trombosit agregasyonunun kalıtımsal bozukluğudur. Glanzmann Trombastenisinin göz bulgularıyla nadiren karşılaşılır. Standart tedavi trombosit transfüzyonudur; bununla birlikte tekrarlanan transfüzyonlar trombosite karşı antikor oluşumu ve trombosit refrakterliği ile sonuçlanmaktadır. Rekombinan aktif faktör VIIa (rFVIIa) trombosit antikoru ve/veya trombosit refrakterliği olan GT hastalarda efektif alternatif bir tedavidir. Burada rFVIIa ile başarılı bir şekilde tedavi edilen travmatik hifeması olan GT’li bir hastayı sunduk.

Anahtar Kelimeler:

Glanzmann trombastenisi, Hifema, Trombosit refrakterliği, Rekombinan faktör VIIa

Traumatic Hyphema Successfully Treated with Recombinant Factor VIIa in Glanzmann Thrombasthenia with Platelet

Glanzmann thrombasthenia (GT) is a congenital disorder of platelet aggregation characterized by a lifelong bleeding tendency. Standard therapy consists of platelet transfusions; however repeated transfusions may result in antiplatelet antibodies and platelet refractoriness. Recombinant activated factor VII (rFVIIa) is an effective alternative therapy in GT patients, particularly in those with antiplatelet antibodies and/or platelet refractoriness. Ocular manifestations of GT are very rare. Here we report a case of GT with traumatic hyphema who was successfully treated with rFVIIa

Keywords:

Glanzmann thrombasthenia, Hyphema, Platelet refractoriness, Recombinant factor VIIa,

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