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

Öz

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

Öz

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

Anahtar Kelimeler

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

Kaynakça

1. Nurden AT. Qualitative disorders of platelets and megakaryocytes. J Thromb Haemost 2005;3:1773-82.
2. Nurden AT. Glanzmann thrombasthenia. Orphanet J Rare Dis 2006;1:10.
3. Hod E, Schwartz J. Platelet transfusion refractoriness. Br J Haematol 2008;142: 348-60.
4. Poon MC. The evidence for the use of recombinant human activated factor VII in the treatment of bleeding patients with quantitative and qualitative platelet disorders. Transfus Med Rev 2007;21:223-36.
5. Maggs PB, MJ, Chalmers EA, Collins PW. A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO. Br J Haematol 2006;135:603-33.
6. Kamburoğlu G, Kiratli H. Recurrent traumatic hyphema in a patient with Glanzmann thrombasthenia. J AAPOS 2005;10:186-7.
7. Slichter SJ. Evidence-based platelet transfusion guidelines. Hematology Am Soc Hematol Educ Program 2007;172-8.
8. Santoro C, Rago A, Biondo F, Conti L, Pulcinelli F, Laurenti L, et al. Prevalence of allo-immunization anti-HLA and anti- integrin alphaIIbbeta3 in Glanzmann Thromboasthenia patients. Haemophilia 2010;16:805-12.

9. Salama ME, Raman S, Drew MJ, Abdel-Raheem M, Mahmood MN. Platelet function testing to assess effectiveness of platelet transfusion therapy. Transfus Apher Sci 2004;30:93-100.
10. Poon MC, d’Oiron R, Zotz RB, Bindslev N, Di Minno MC, Di Minno G. The international, prospective Glanzmann Thrombasthenia Registry: Treatment and outcomes in surgical intervention Haematologica 2015;100:1038-44.
11. Coller BS, French DL. Hereditary qualitative platelet disorders. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U (eds). Williams Hematology, 6th ed. New York: McGraw-Hill, 2001: 1551- 60.
12. Galan AM, Tonda R, Pino M, Reverter JC, Ordinas A, Escolar G. Increased local procoagulant action: A mechanism contributing to the favorable hemostatic effect of recombinant FVIIa in PLT disorders. Transfusion 2003;43:885-92.
13. Nurden AT, Pillois X, Wilcox DA. Glanzmann thrombasthenia: State of the art and future directions. Semin Thromb Hemost 2013;39:642–55.
14. Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: Pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med 2015;6:219-27.