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.

Kaynakça

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