[Safety of use of velaglucerase in 2 patients with type 1 Gaucher's disease].
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.
Atención Primaria Delicias Norte, Fundación Española para el Estudio y Tratamiento de la Enfermedad de Gaucher, Zaragoza, Spain. platre05@gmail.com
Paz
P
Latre
Pilar
P
Giraldo
D002675:Child, Preschool; D056947:Enzyme Replacement Therapy / Q000009:adverse effects; D005260:Female; D005776:Gaucher Disease / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D005962:Glucosylceramidase / Q000009:adverse effects / Q000276:immunology / Q000627:therapeutic use*; D006801:Humans; D011994:Recombinant Proteins / Q000009:adverse effects / Q000276:immunology
D011994:Recombinant Proteins; D005962:Glucosylceramidase; C550184:Velaglucerase alfa, human; C090568:imiglucerase