Rituximab and desensitization for a patient with severe factor IX deficiency, inhibitors, and history of anaphylaxis

J Pediatr Hematol Oncol. 2008 Jan;30(1):93-5. doi: 10.1097/MPH.0b013e31815cf742.

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.

Publication types

  • Case Reports

MeSH terms

  • Anaphylaxis / chemically induced
  • Anaphylaxis / drug therapy*
  • Anaphylaxis / immunology
  • Antibodies, Monoclonal / administration & dosage*
  • Antibodies, Monoclonal, Murine-Derived
  • Blood Coagulation Factor Inhibitors / immunology*
  • Child
  • Desensitization, Immunologic*
  • Factor IX / administration & dosage*
  • Factor IX / adverse effects*
  • Factor IX / immunology
  • Hemophilia B / complications
  • Hemophilia B / drug therapy*
  • Hemophilia B / immunology
  • Humans
  • Immunologic Factors / administration & dosage*
  • Kinetics
  • Male
  • Recombinant Proteins / administration & dosage
  • Recombinant Proteins / adverse effects
  • Rituximab
  • Time Factors

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Blood Coagulation Factor Inhibitors
  • Immunologic Factors
  • Recombinant Proteins
  • Rituximab
  • Factor IX