Idursulfase desensitization in a child with Hunter syndrome (mucopolysaccharidosis II)

Abstract

Enzyme replacement therapy with idursulfase decreases morbidity and improves quality of life of patients with mucopolysaccharidosisii.Immediatehypersensitivityreactions tothisdrughavebeendescribed.Desensitizationisatreatment that induces temporary tolerance to a culprit drug, allowing the allergic patient to receive the medication. We present the case of a 7-year-old patient diagnosed with Hunter syndrome who presented, after 4 years of treatment, twoepisodesofanaphylaxisduringtheinfusionofidursulfase. Detection of specific immunoglobulin E was carried out using skintests,withintradermalreactionata1/10dilution(0.2mg/ml) being positive. A 12-step desensitization protocol was performed without presenting adverse events. The allergological evaluation and the possibility of desensitization were useful tools in the management of our patient.