title: Rituximab and desensitization for a patient with severe factor IX deficiency, inhibitors, and history of anaphylaxis.
abstract: We report the case of a 9-year-old boy with severe hemophilia B with an inhibitor and a history of anaphylaxis to factor IX (FIX), who was successfully treated with immune tolerance (IT) that included rituximab (Genentech, San Francisco, CA) and desensitization. The patient began the first course of IT at age of 7 years with daily recombinant FIX infusions that was aborted at day 32 because of a systemic allergic reaction. A second course of IT was attempted 11 months later, initiated with a desensitization protocol with escalating doses of plasma-derived FIX in addition to therapy with rituximab. This was well tolerated without allergic symptoms and with eradication of the inhibitor and improvement in factor kinetics. A second course of rituximab was given 13 months later for worsening FIX kinetics again with an excellent response and without evidence of adverse effects.
authors.affiliation: Division of Paediatric Haematology, University of Toronto, Toronto, Ontario, Canada. Sarah.Alexander@sickkids.ca
authors.forename: Sarah
authors.initials: S
authors.lastname: Alexander
authors.forename: Steve
authors.initials: S
authors.lastname: Hopewell
authors.forename: Susan
authors.initials: S
authors.lastname: Hunter
authors.forename: Akhilesh
authors.initials: A
authors.lastname: Chouksey
mesh_terms: D000707:Anaphylaxis / Q000139:chemically induced / Q000188:drug therapy* / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D019774:Blood Coagulation Factor Inhibitors / Q000276:immunology*; D002648:Child; D003888:Desensitization, Immunologic; D005164:Factor IX / Q000008:administration & dosage* / Q000009:adverse effects* / Q000276:immunology; D002836:Hemophilia B / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D007700:Kinetics; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000009:adverse effects; D000069283:Rituximab; D013997:Time Factors
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D019774:Blood Coagulation Factor Inhibitors; D007155:Immunologic Factors; D011994:Recombinant Proteins; D000069283:Rituximab; D005164:Factor IX