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

The most effective treatment strategy for patients with hemophilia is replacement therapy with FVIII or FIXconcentrates, which usually requires long-term, uncomplicated venous access. However, central venous access device (CVADs, ports) insertion requires inpatient admission and general anesthesia, and presents some problems regarding health insurance coverage. Peripherally inserted central catheters (PICCs) were inserted in two severe hemophilia patients aged 7 and 11 years with high titers of inhibitors. They experienced frequent bleeding episodes and required replacement therapy, which eventually resulted in difficulty in acquiring venous line access. Factor VIII activity was below 1%, and inhibitor titers were 160 and 26.3 BU/ml. In an outpatient setting, PICC lines are easily placed by radiological guidance and require local anesthesia alone. PICC has been feasible, in particular, for hemophilia patients with frequent bleeding episodes.
Aged ; Anesthesia, General ; Anesthesia, Local ; Catheters ; Factor VIII ; Hemophilia A ; Hemorrhage ; Humans ; Inpatients ; Insurance, Health ; Outpatients