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Induction of immune tolerance and suppression of anaphylaxis in a child with haemophilia B by simple plasmapheresis and antigen exposure
Author(s) -
Barnes C.,
Rudzki Z.,
Ekert H.
Publication year - 2000
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1046/j.1365-2516.2000.00419.x
Subject(s) - plasmapheresis , medicine , haemophilia b , haemophilia , anaphylaxis , immunology , haemophilia a , antigen , immune tolerance , pediatrics , allergy , antibody
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.