SUCCESSFUL IMIGLUCERASE DESENSITIZATION IN A PATIENT WITH SEVERE ARTHRALGIA AND MYALGIA

Abstract

<h3>Introduction</h3> A patient with Type I Gaucher disease experienced severe musculoskeletal pain within minutes upon first and second administration of imiglucerase, which is a rare adverse event. He underwent successful desensitization, which has only been reported in 3 cases, and continues to receive imiglucerase therapy. <h3>Case Description</h3> A 37-year-old male with Type I Gaucher disease developed severe arthralgias and myalgias without skin changes nor symptoms of anaphylaxis within 5 minutes of the first infusion of imiglucerase enzyme replacement therapy. The infusion was stopped, and he was treated with intravenous (IV) fluids, IV hydrocortisone, and acetaminophen. He experienced severe back pain at a second infusion a week later despite pretreatment with IV diphenhydramine and prednisolone. A desensitization procedure was performed using an intermediate risk (3 bag) protocol (44U, followed by 440U and 4400U). Premedication included intravenous (IV) dexamethasone twelve hours in advance, and IV dexamethasone, aspirin 325mg, IV diphenhydramine, and IV famotidine 30 minutes prior to the first bag. He tolerated the desensitization protocol without a reaction and has progressed to monthly infusions with pretreatment without adverse reactions. <h3>Discussion</h3> Adverse reactions to imiglucerase are an infrequent setback in Gaucher disease treatment, with mild cutaneous or respiratory symptoms primarily reported resolving with slowed infusion rates. Although hypersensitivity reactions suspected to be IgG-mediated have been described within the first six months of therapy, this case represents an even more rare immediate muscular hypersensitivity reaction upon first imiglucerase exposure with a successful model for desensitization and continued current tolerance of active therapy.