Rapid Desensitization for Immediate Hypersensitivi

Authors: Zeynep Tamay Gulden Gokcay Fatih Dilek Mehmet Cihan Balci Deniz Ozceker Mubeccel Demirkol Nermin Guler

Publish Date: 2016

Volume: , Issue: , Pages: 53-57

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Abstract

Mucopolysaccharidosis type VI MPS VI is a progressive chronic and multisystem lysosomal storage disease Enzyme replacement therapy ERT with the recombinant human arylsulfatase B enzyme galsulfase Naglazyme is recommended as firstline therapy It is generally reported as safe and well tolerated Frequently observed mild to moderate infusionrelated reactions which can be easily handled by reducing or interrupting the infusion and/or administering additional antihistamines antipyretics and corticosteroids are mostly mediated by nonIgE mechanisms Here we report two children with MPS VI who experienced IgEmediated reactions with galsulfase at the second year of the therapy One child had anaphylaxis and the other had urticarial eruptions They could receive ERT after successful rapid desensitization To our knowledge this is the second report on galsulfase allergy with IgEmediated reaction It is important to recognize IgEmediated reactions since they can be lifethreatening and do not respond to the standard therapies We recommend allergy skin tests in the evaluation of infusionrelated reactions unresponsive to standard therapies so that continuation of ERT will be feasible after successful desensitizationAll procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation institutional and national and with the Helsinki Declaration of 1975 as revised in 2000 Informed consent was obtained from all patients for being included in the studyZeynep Tamay designed and reported the cases Fatih Dilek Mehmet C Balci and Deniz Ozceker conducted the rapid desensitization procedures Gulden Gokcay revised the language and also revised the intellectual content with Mubeccel Demirkol and Nermin Guler

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