INHIBITOR PADA HEMOFILIA

ABSTRAK Hemofilia adalah penyakit perdarahan akibat kelainan faal koagulasi yang bersifat herediter, diturunkan secara X-linked recessive dengan prevalensi 5000-10.000 penduduk laki-laki yang lahir hidup. Penderita dengan hemofilia A, B atau C, inhibitor langsung melawan faktor VIII, IX atau XI selama diberikan terapi pengganti. Insiden terbentuknya inhibitor pada hemofilia berkisar 20-40%, sedangkan pada penderita hemofilia B berkisar 1-6%. Pemeriksaan faktor inhibitor secara kuantitatif menggunakan Bethesda inhibitor assay. Terapi hemofilia dengan inhibitor masih merupakan tantangan dan memerlukan pengalaman. Pada pasien hemofilia, terbentuknya inhibitor akan memperburuk prognosis. (MEDICINA 2012;43:31-36). Kata kunci: hemofilia, inhibitor, faktor VIII INHIBITOR IN HEMOPHILIA Veny K Yantie, Ariawati K Department of Child Health, Medical School, Udayana University/Sanglah Hospital , Denpasar ABSTRACT Hemophilia is an X-linked recessive disorder which is believed to affect approximately one in 5000-10.000 male birth. An inhibitor is a type of antibody. In hemophilia patients type A, B, and C are directly destroy factor VII, IX, and XI. The incidence of antibody development in hemophilia A is between 20% and 40%, hemophilia B inhibitors only 1 to 6 %. The presence of an inhibitor is usually confirmed using a specific blood test called the Bethesda inhibitor assay. The treatment of hemophilic bleeding in a person with an inhibitor can be a challenging experience. Patients hemophilia with inhibitor have poor prognostic. (MEDICINA 2012;43:31-36). Keywords: hemophillia, inhibitor, factor VIII