Successful desensitization to imiglucerase of an adult patient diagnosed with type I Gaucher disease

Int Arch Allergy Immunol. 2013;160(2):215-7. doi: 10.1159/000339754. Epub 2012 Sep 25.


Gaucher disease is the most common lysosomal storage disorder, and enzyme replacement therapy, such as administration of imiglucerase, is the standard therapy. Anaphylaxis to imiglucerase is rarely reported. Here, we report a 26-year-old female who was diagnosed with type 1 Gaucher disease and referred to our Allergy Outpatient Clinic because of an anaphylactic reaction due to imiglucerase enzyme therapy. A desensitization protocol was administered with two different dilutions with an increasing rate of administration delivered in 10 consecutive steps by intravenous infusion in an intensive care setting. No reactions occurred during the procedure, and the total final dose of 2,000 U was successfully administered. To our knowledge, this is the first adult case with successful desensitization to imiglucerase. Desensitization protocols to drugs in chronic disease patients for whom no alternative therapies are available can be lifesaving.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anaphylaxis / immunology
  • Anaphylaxis / prevention & control*
  • Desensitization, Immunologic / methods*
  • Drug Hypersensitivity / immunology*
  • Drug Hypersensitivity / prevention & control*
  • Enzyme Replacement Therapy / adverse effects*
  • Female
  • Gaucher Disease / drug therapy
  • Gaucher Disease / immunology*
  • Glucosylceramidase / adverse effects
  • Glucosylceramidase / immunology*
  • Glucosylceramidase / therapeutic use
  • Humans


  • Glucosylceramidase
  • imiglucerase