eliglustat-tartrate and Anaphylaxis

Topics: Adult; alpha-Galactosidase; Anaphylaxis; Desensitization, Immunologic; Drug Hypersensitivity; Enzyme Replacement Therapy; Fabry Disease; Humans; Isoenzymes; Male; Omalizumab; Treatment Outcome

Topics: alpha-Galactosidase; Anaphylaxis; Desensitization, Immunologic; Enzyme Replacement Therapy; Fabry Disease; Humans; Immunoglobulin E; Immunoglobulin G; Isoenzymes; Kidney Transplantation; Male; Middle Aged; Tacrolimus

To report a severe adverse event related to enzyme replacement therapy with agalsidase in an hemizygous male patient treated for Fabry disease.. Retrospective analysis of clinical, radiological and biochemical data in a patient who suffered adverse events related to both agalsidase alfa and agalsidase beta treatments.. A hemizygous male patient was first treated for Fabry disease with agalsidase alfa. After more than 1 year of therapy, infusion-related symptoms necessitated systemic steroids and antihistaminic therapy. Decline in kidney function prompted a switch for agalsidase beta. Anaphylactoid shock occurred after the second infusion. No serum IgE antibodies were disclosed. Skin-test reactivity to agalsidase beta was negative. Following a published rechallenge infusion protocol, agalsidase beta was reintroduced, leading to a second anaphylactoid shock episode. Enzyme replacement therapy was stopped and the patient was treated with symptomatic therapy only. This case was referred to the pharmacovigilance department.. The negativity of immunological tests (specific anti-agalsidase IgE antibodies and skin tests) does not rule out the risk of repeated anaphylactoid shock following agalsidase infusion.

Topics: Adult; alpha-Galactosidase; Anaphylaxis; Antibodies, Anti-Idiotypic; Enzyme Replacement Therapy; Fabry Disease; Humans; Isoenzymes; Male; Recombinant Proteins; Risk Factors; Treatment Outcome