title: Successful desensitization to imiglucerase of an adult patient diagnosed with type I Gaucher disease.
abstract: 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.
authors.affiliation: Division of Allergy, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Derya
authors.initials: D
authors.lastname: Erdoğdu
authors.forename: Aslı
authors.initials: A
authors.lastname: Gelincik
authors.forename: Bülent
authors.initials: B
authors.lastname: Canbaz
authors.forename: Bahattin
authors.initials: B
authors.lastname: Colakoğlu
authors.forename: Suna
authors.initials: S
authors.lastname: Büyüköztürk
authors.forename: Refik
authors.initials: R
authors.lastname: Tanakol
mesh_terms: D000328:Adult; D000707:Anaphylaxis / Q000276:immunology / Q000517:prevention & control*; D003888:Desensitization, Immunologic / Q000379:methods*; D004342:Drug Hypersensitivity / Q000276:immunology* / Q000517:prevention & control*; D056947:Enzyme Replacement Therapy / Q000009:adverse effects*; D005260:Female; D005776:Gaucher Disease / Q000188:drug therapy / Q000276:immunology*; D005962:Glucosylceramidase / Q000009:adverse effects / Q000276:immunology* / Q000627:therapeutic use; D006801:Humans
chemical_list: D005962:Glucosylceramidase; C090568:imiglucerase