[Safety of use of velaglucerase in 2 patients with type 1 Gaucher's disease]

Med Clin (Barc). 2011 Sep:137 Suppl 1:39-42. doi: 10.1016/S0025-7753(11)70015-X.
[Article in Spanish]

Abstract

The incidence of immunologic reactions in patients with Gaucher's disease (GD) on enzymatic replacement therapy with imiglucerase is about 17% and related with the presence of non-neutralizing immunoglobulin G antibodies. The clinical trials with a new enzyme obtained in human cells (GA-GCB-velaglucerase) have demonstrated absence of immune reactions and no antibodies against the enzyme in spite of some patients had previous developed antibodies against imiglucerase. We present 2 clinical cases of patients diagnosed with EG in childhood and who developed antibodies and important imiglucerase immunoallergic adverse reaction during the imiglucerase perfusion, indicating systematic administration of steroids and antihistamines prior to each perfusion and perfusion time > 4h.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Enzyme Replacement Therapy* / adverse effects
  • Female
  • Gaucher Disease / diagnosis
  • Gaucher Disease / drug therapy*
  • Gaucher Disease / genetics
  • Glucosylceramidase / adverse effects
  • Glucosylceramidase / immunology
  • Glucosylceramidase / therapeutic use*
  • Humans
  • Recombinant Proteins / adverse effects
  • Recombinant Proteins / immunology

Substances

  • Recombinant Proteins
  • Glucosylceramidase
  • Velaglucerase alfa, human
  • imiglucerase