• Pol. Merkur. Lekarski · Mar 2011

    Review

    [Prophylaxis in patients with haemophilia complicated by inhibitors].

    • Łaguna Paweł.
    • Katedra i Klinika Pediatrii Hematologii i Onkologii Warszawskiego Uniwersytetu Medycznego. skazy@litewska.edu.pl
    • Pol. Merkur. Lekarski. 2011 Mar 1; 30 (177): 211-4.

    AbstractThe occurrence of factor VIII or factor IX inhibitor following treatment of a haemophilia A or B patient with factor VIII/IX concentrates is a serious complication. In the presence of these inhibitors, bleeds cannot be treated with the missing factors. The treatment of haemophilia complicated by the presence of inhibitor has two aims. The most important aim is complete elimination of the inhibitor, and the other aim is to stop bleeding. The first aim can be achieved by regular administration of the missing factor in order to eliminate inhibitor from the patient's blood. This method is called--immune tolerance induction (ITI) The second method depends on the titer of the inhibitor, types of response and degree of bleeding. In treatment of bleeding episodes concentrates inducing thrombin generation in the plasma of patients with haemophilia complicated by inhibitor are used. This assures haemostasis, in spite of the presence of factor VIII or IX inhibitor. The concentrates used are called bypassing agents. If ITI is unsuccessful regular prophylaxis with bypassing concentrates is being introduced more and more often. According to literature this reduces the frequency of bleeds by as much as 85% and improves the quality of life of patients with severe haemophilia complicated by inhibitor.

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