Elif SELVIOĞLU, Merve BIYIKLI, Emine Esra GÜNER, İrfan YAVAŞOĞLU

Edinsel hemofili A deneyimi

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.

Experience of acquired haemophilia A

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.

PDF

___

1. Franchini M, Targher G, Montagnana M, Lippi G. Laboratory, clinical and therapeutic aspects of acquired hemophilia A. Clin Chim Acta 2008;395:14-18. https:// doi.org/10.1016/j.cca.2008.05.003
2. Collins P, Macartney N, Davies R, Lees S, Giddings J, Majer R. A population based, unselected, consecutive cohort of patients with acquired hemophilia A. Br J Haematol 2004;124:86-90. https://doi.org/10.1046/ j.1365-2141.2003.04731.x
3. Collins P, Hirsch S, Baglin TP, 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- 1877. https://doi.org/10.1182/blood-2006-06-029850
4. Ege B, Geyik A, Utkun M. Hemofili A tanısı almış bir hastada meydana gelmiş odontojenik apsenin tedavisi. J Biotechnol and Strategic Health Res 2018;2:205-211.
5. Hay CR. Factor VIII inhibitors. Haemophilia. 1995;2:14- 21. https://doi.org/10.1111/j.1365-2516.1995.tb00117.x
6. Fosbury E, Drebes A, Riddell A, Chowdary P. Review of recombinant anti-haemophilic porcine sequence factor VIII in adults with acquired haemophilia A. Ther Adv Hematol 2017;8:263-272. https://doi. org/10.1177/2040620717720861
7. Kruse Jarres R, Kempton CL, Baudo F, et al. Acquired hemophilia A: updated review of evidence and treatment guidance. Am J Hematol 2017;92:695-705. https://doi.org/10.1002/ajh.24777
8. Bitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis 2009;20:517-523. https://doi.org/10.1097/ MBC.0b013e32832ca388
9. Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol 2005;80:55-63. https://doi.org/10.1002/ajh.20390
10. Delgado J, Jimenez Yuste V, Hernandez Navarro F, Villar A. Acquired haemophilia: review and metaanalysis focused on therapy and prognostic factors. Br J Haematol 2003;121:21-35. https://doi.org/10.1046/ j.1365-2141.2003.04162.x
11. Franchini M, Castaman G, Coppola A, et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus. 2015;13:498-513. https://doi.org/10.2450/2015.0141- 15
12. Franchini M, Vaglio S, Marano G, et al. Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology 2017;22:514-520. https://doi.org/10.1080/10245332.2017.1319115
13. Zeng Y. et al. Rituximab for eradicating inhibitors in people with acquired haemophilia A. Cochrane Database Syst Rev 2016;7:011907. https://doi. org/10.1002/14651858.CD011907.pub2