Rapid Desensitization for Immediate Hypersensitivity to Galsulfase Therapy in Patients with MPS VI

  • Zeynep TamayEmail author
  • Gulden Gokcay
  • Fatih Dilek
  • Mehmet Cihan Balci
  • Deniz Ozceker
  • Mubeccel Demirkol
  • Nermin Guler
Case Report
Part of the JIMD Reports book series (JIMD, volume 30)


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 first-line therapy. It is generally reported as safe and well tolerated. Frequently observed mild to moderate infusion-related reactions which can be easily handled by reducing or interrupting the infusion and/or administering additional antihistamines, antipyretics, and corticosteroids are mostly mediated by non-IgE mechanisms. Here we report two children with MPS VI who experienced IgE-mediated 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 IgE-mediated reaction. It is important to recognize IgE-mediated reactions since they can be life-threatening and do not respond to the standard therapies. We recommend allergy skin tests in the evaluation of infusion-related reactions unresponsive to standard therapies, so that continuation of ERT will be feasible after successful desensitization.


Enzyme Replacement Therapy Intradermal Test Desensitization Protocol Desensitization Procedure Rapid Desensitization 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Brigham and Women’s Hospital


Enzyme replacement therapy




Mucopolysaccharidosis type VI


Arylsulfatase B


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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Zeynep Tamay
    • 1
    Email author
  • Gulden Gokcay
    • 3
  • Fatih Dilek
    • 4
  • Mehmet Cihan Balci
    • 3
  • Deniz Ozceker
    • 1
  • Mubeccel Demirkol
    • 3
  • Nermin Guler
    • 2
  1. 1.Division of Pediatric Allergy and Clinical Immunology, Department of PediatricsIstanbul Medical FacultyIstanbulTurkey
  2. 2.Department of PediatricsFaculty of Medicine, Istanbul Bilim UniversityIstanbulTurkey
  3. 3.Division of Pediatric Nutrition and Metabolism, Department of PediatricsIstanbul Medical FacultyIstanbulTurkey
  4. 4.Division of Pediatric Allergy and Clinical Immunology, Department of PediatricsBezmialem Vakıf UniversityIstanbulTurkey

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