Gri trombosit sendromu
Gri trombosit (platelet) sendromu (GPS), trombositopeni ve ışık mikroskopunda soluk görünen kusurlu trombositlerle karakterize, otozomal resesif geçişli bir hastalıktır. Hastalarda kolay morarma, burun kanaması, menoraji ve uzun kanamalar görülmektedir. GPS için spesifik bir tedavi bulunmamaktadır dolayısı ile hastalığa karşı, riskleri öngörmek ve kanamanın önlenmesi için trombosit fonksiyonunu bozan ilaçlardan kaçınmak gerekmektedir. Bu olgu sunumunda, tekrarlayan anormal kanama atakları olan ve GPS bulgusu bulunan bir vaka sunulmaktadır.
Gray platelet syndrome
Gray platelet syndrome (GPS) is an autosomal recessive disorder characterized by thrombocytopenia and defective platelets that appear pale on light microscope. Patients present with easy bruisability, nose bleeds, menorrhagia and prolonged bleeding. There is no specific treatment for GPS and the management includes anticipating risks and preventing bleeding by avoiding drugs that impair platelet function. We present here report of a case who presented with repeated episodes of abnormal bleeding and was found to have GPS.
___
- 1. Raccuglia G. Gray platelet syndrome. A variety of qualitative platelet disorder. Am J Med. 1971;51:818-28.
- 2. Balduini CL, Iolascon A, Savoia A. Inherited thrombocytopenias: from genes to therapy. Haematologica. 2002;87:860-80.
- 3. Laffan M, Manning R. Investigation of haemostasis. In Practical Hematology, 10th ed (Eds Dacie JV, Lewis SM):429. Philadelphia, PA, Elsevier. 2006.
- 4. Nurden AT, Nurden P. The gray platelet syndrome: clinical spectrum of the disease. Blood Rev. 2007;21:21-36.
- 5. Nurden AT, Nurden P. Inherited thrombocytopenias. Haematologica. 2007;92:1158-64.
- 6. Online Mendelian inheritance in man. http://omim.org (accessed 23 Oct 2015).
- 7. Nurden P, Nurden AT. Congenital disorders associated with platelet dysfunctions. Thromb Haemost. 2008;99:253-63.
- 8. Salles II, Feys HB, Iserbyt BF, De Meyer SF, Vanhoorelbeke K, Deckmyn H. Inherited traits affecting platelet function. Blood Rev. 2008;22:155-72.
- 9. Drachman JG. Inherited thrombocytopenia: when a low platelet count does not mean ITP. Blood. 2004;103:390-8.
- 10. Laskey AL, Tobias JD; Anesthetic implications of the gray platelet syndrome. Can J Anaesth. 2000;47:1224-9.
- 11. Balduini CL, Iolascon A, Savoia A. Inherited thrombocytopenias: from genes to therapy. Haematologica. 2002;87:860-80.
- 12. Mannucci PM. Prophylaxis in congenital coagulation disorders: past, present and future. Blood Transfus. 2008;6:1-3.