Agalsidase-α/agalsidase-β
Transcript of 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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