Journal List > Korean J Hematol > v.43(3) > 1032782

Park and Yoo: A Case of Desensitization for Hemophilia B Inhibitor Patient with Anaphylaxis to FIX Concentrates

Abstract

Among the patients with hemophilia, 10∼15% have hemophilia B, and 1∼3% of the hemophilia B patients develop inhibitor to factor IX clotting activity. Allergic reactions to concentrates containing factor IX (FIX) are serious complications during the treatment of hemophilia B patients with inhibitor. Although treatment with recombinant activated factor VII (FVIIa) is generally recommended in these patients, it is limited by the agent's short half-life, a lack of experience with its use in this manner and the prohibitive cost. We report here on a case of a 9-year-old boy with severe hemophilia B with inhibitor and he had a history of anaphylaxis to FIX. The patient was successfully treated with a desensitization protocol with escalating doses of FIX in addition to administering premedications.

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Fig. 1
The inhibitor titer during desensitization therapy.
kjh-43-179f1.tif
Table 1.
Protocol and response for the treatment of hemophilia B inhibitor patient with anaphylactic reactions
Medication Benefix             FEIBA  
Dose (u) 50 150 300 500 750 1,000 1,500 2,000 2,000
Anaphylactic reaction + +/−
Factor IX antibody (BU) <1 <1   <1   45 155    
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