Edinsel hemofili A deneyimi.

Öz

Edinsel hemofili A, hemofili aile öyküsü olmayan bireylerde endojen faktör VIII (FVIII)’e karşı gelişen oto antikorlar sebebiyle oluşan nadir bir kanama bozukluğudur. İnsidansı milyonda 1,30-1,50 olarak bildirilmiştir. Klinik olarak spontan kanama riskinin yüksek olduğu ciddi hemofili A’ya benzer ancak kanamalar daha çok mukokütanöz, yumuşak doku veya gastrointestinal kanama şeklindedir. İnhibitör gelişimi Hemofili A vakalarında önemli sorunlardan biridir. Edinsel hemofili A tedavisinde birinci seçenek tedaviler baypas yapıcı ajanlardır. Bu amaçla rekombinant faktör VIIa ve aktive protombin kompleks konsantreleri kullanılmaktadır. Burada edinsel hemofili A ve faktör VIII inhibitör pozitifliği olan bir olgu sunulmuştur.

Anahtar Kelimeler

• Faktör VIII
• inhibitör
• hemofili A
• edinsel

Experience of acquıred haemophilia A.

Abstract

Summary: Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to endogenous factor VIII (FVIII) in individuals without a family history of hemophilia. Its incidence is reported to be 1.30-1.50 per million. Clinically it is similar to severe hemophilia A, where the risk of spontaneous bleeding is high, but bleeding is mostly mucocutaneous, soft tissue or gastrointestinal bleeding. Inhibitor development is one of the major problems in hemophilia A cases. Bypassing agents are the first-line treatments for acquired hemophilia A. For this purpose, recombinant factor VIIa and activated protombin complex concentrates are used. We present a case with acquired Hemophilia A and factor VIII inhibitor positivity.

Keywords

• Factor VIII
• inhibitor
• hemophilia A
• acquired

References

1. 1) Franchini M et al. Laboratory, clinical and therapeutic aspects of acquired hemophilia A. Clin Chim Acta 2008; 395: 14-8. 2) Collins P et al. A population based, unselected, consecutivecohort of patients with acquired hemophilia A. Br J Haematol 2004;124:86-90. 3) Collins P et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood 2007; 109: 1870-7. 4) J Biotechnol and Strategic Health Res. 2018;2(3):205-211 5) Hay CR. Factor VIII inhibitors. Haemophilia, 1995; 1(suppl 3): 14-21. 6) Ther Adv Hematol. 2017 Sep; 8(9): 263–272. 7) Kruse‐Jarres R et al.: Acquired hemophilia A: updated review of evidence and treatment guidance. Am J Hematol 2017; 92: 695– 705 8) Bitting RL et al. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis 2009;20:517-523 9) Franchini M et al. Acquired hemophilia A: a concise review. Am J Hematol 2005;80:55-63. 10) Delgado J et al. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003;121:21-35. 11) Franchini M et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus. 2015;13:498–513. 12) Franchini, M et al. Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology. 2017; 22: 514–520 13) Zeng Y et al. Rituximab for eradicating inhibitors in people with acquired haemophilia A. Cochrane Database Syst Rev. 2016;7(7):011907