Korean J Hematol.  2008 Sep;43(3):179-183. 10.5045/kjh.2008.43.3.179.

A Case of Desensitization for Hemophilia B Inhibitor Patient with Anaphylaxis to FIX Concentrates

• Park Y
• Yoo KY

• KMID: 2252186
• DOI: http://doi.org/10.5045/kjh.2008.43.3.179

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.

Keyword

Hemophilia B; Inhibitor; Anaphylaxis; Desensitization

MeSH Terms

Anaphylaxis
Child
Factor IX
Factor VIIa
Half-Life
Hemophilia A
Hemophilia B
Humans
Hypersensitivity
Factor IX
Factor VIIa