Agalsidase-α/agalsidase-β

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Reactions 1491, p6 - 8 Mar 2014 Agalsidase-α/agalsidase-β Infusion associated reactions: 3 case reports A retrospective study identified two male patients and one female patient [ages not stated] with anti-agalsidase antibody- negative Fabry disease who developed infusion associated reactions * during treatment with agalsidase-α (one patient) or agalsidase-β (two patients). A male patient received IV agalsidase-β 1.0 mg/kg every other week. After 56 weeks, he developed chills and fever. He received dexamethasone pretreatment before his next infusions; infusion associated reactions not reoccur. A male patient received IV agalsidase-α 0.2 mg/kg every other week. He developed recurrent shivers and sensation of fever [duration of treatment to reaction onset and outcome not stated]. A female patient received IV agalsidase-β 0.2 mg/kg every other week. She developed the sensation of fever [duration of treatment to reaction onset not stated]. Her infusion rate was lowered [outcome not stated]. Author comment: "Three AB– [anti-agalsidase antibody- negative] patients experienced IARs [immune associated reactions] (chills, shivers and fever) in which a relation to IgG antibodies was considered." * * Overall, 17 antibody-positive and 16 antibody-negative patients developed immune associated reactions; however, individual patient details were not provided for the remaining patients. Smid BE, et al. A revised home treatment algorithm for Fabry disease: Influence of antibody formation. Molecular Genetics and Metabolism 108: 132-137, No. 2, Feb 2013 - Netherlands 803099979 1 Reactions 8 Mar 2014 No. 1491 0114-9954/14/1491-0001/$14.95 Adis © 2014 Springer International Publishing AG. All rights reserved

Transcript of Agalsidase-α/agalsidase-β

Page 1: Agalsidase-α/agalsidase-β

Reactions 1491, p6 - 8 Mar 2014

Agalsidase-α/agalsidase-βInfusion associated reactions: 3 case reports

A retrospective study identified two male patients and onefemale patient [ages not stated] with anti-agalsidase antibody-negative Fabry disease who developed infusion associatedreactions* during treatment with agalsidase-α (one patient) oragalsidase-β (two patients).

A male patient received IV agalsidase-β 1.0 mg/kg everyother week. After 56 weeks, he developed chills and fever. Hereceived dexamethasone pretreatment before his nextinfusions; infusion associated reactions not reoccur.

A male patient received IV agalsidase-α 0.2 mg/kg everyother week. He developed recurrent shivers and sensation offever [duration of treatment to reaction onset and outcome notstated].

A female patient received IV agalsidase-β 0.2 mg/kg everyother week. She developed the sensation of fever [duration oftreatment to reaction onset not stated]. Her infusion rate waslowered [outcome not stated].

Author comment: "Three AB– [anti-agalsidase antibody-negative] patients experienced IARs [immune associatedreactions] (chills, shivers and fever) in which a relation to IgGantibodies was considered."

* * Overall, 17 antibody-positive and 16 antibody-negative patientsdeveloped immune associated reactions; however, individual patientdetails were not provided for the remaining patients.

Smid BE, et al. A revised home treatment algorithm for Fabry disease: Influenceof antibody formation. Molecular Genetics and Metabolism 108: 132-137, No. 2,Feb 2013 - Netherlands 803099979

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Reactions 8 Mar 2014 No. 14910114-9954/14/1491-0001/$14.95 Adis © 2014 Springer International Publishing AG. All rights reserved