Autor: |
C, Barnes, Z, Rudzki, H, Ekert |
Rok vydání: |
2000 |
Předmět: |
|
Zdroj: |
Haemophilia : the official journal of the World Federation of Hemophilia. 6(6) |
ISSN: |
1351-8216 |
Popis: |
Anaphylaxis to factor IX (FIX) in patients with haemophilia B is a rare and life-threatening complication that has been reported to occur in association with the development of inhibitors to FIX. Management of these patients is difficult. This report presents an 18-month-old boy with a frame-shift mutation of the FIX gene and FIX coagulant level of1% who developed anaphylactoid reactions to low and high purity plasma-derived FIX concentration infusions and an inhibitor measuring 1.0 BU mL(-1). The patient was managed with simple plasmapheresis, a short course of corticosteroids and high-dose antigen exposure, which successfully induced long-lasting immune tolerance to FIX concentrates. |
Databáze: |
OpenAIRE |
Externí odkaz: |
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