Induction of immune tolerance and suppression of anaphylaxis in a child with haemophilia B by simple plasmapheresis and antigen exposure.
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 of <1% 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.
Barnes
C
C
Department of Haematology and Oncology, Royal Children's Hospital, Flemington Road, Parkville, Victoria 3052, Australia
Rudzki
Z
Z
Ekert
H
H
D000707:Anaphylaxis / Q000209:etiology / Q000276:immunology* / Q000517:prevention & control*; D000941:Antigens / Q000276:immunology*; D005164:Factor IX / Q000008:administration & dosage / Q000009:adverse effects / Q000276:immunology*; D002836:Hemophilia B / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007108:Immune Tolerance; D007223:Infant; D008297:Male; D010956:Plasmapheresis
D000941:Antigens; D005164:Factor IX