A Case of Desensitization for Hemophilia B Inhibitor Patient with Anaphylaxis to FIX Concentrates.
10.5045/kjh.2008.43.3.179
- Author:
Youngshil PARK
1
;
Ki Young YOO
Author Information
1. Department of Pediatrics, Colllege of Medicine, Kyung Hee University, Seoul, Korea. pysmd@khnmc.or.kr
- Publication Type:Case Report
- Keywords:
Hemophilia B;
Inhibitor;
Anaphylaxis;
Desensitization
- MeSH:
Anaphylaxis;
Child;
Factor IX;
Factor VIIa;
Half-Life;
Hemophilia A;
Hemophilia B;
Humans;
Hypersensitivity
- From:Korean Journal of Hematology
2008;43(3):179-183
- CountryRepublic of Korea
- Language:Korean
-
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.
