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

Affiliations
  • 1Department of Pediatrics, Colllege of Medicine, Kyung Hee University, Seoul, Korea. pysmd@khnmc.or.kr
  • 2Korea Hemophilia Foundation, Seoul, Korea.

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

Figure

  • Fig. 1 The inhibitor titer during desensitization therapy.


Cited by  1 articles

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