Successful Desensitization Of A Fabry ’s Patient with Agalsidase-Beta (Fabrazyme™) Anaphylaxis After Omalizumab Pre-Treatment

Fabry disease is a rare lysosomal storage X-linked genetic disorder caused by pathogenic variants in the α-galactosidase A gene (α-Gal A) resulting glycolipid accumulation (mainly globotriaosylceramide or globotriaosylsphingosine) within cell lysosomes. Two Fabry Disease phenotypes in males include a severe form (1:22,570 males) characterized by reduced α-Gal A activity and accumulation of Gb3 in va scular endothelial cells, cardiomyocytes, smooth muscle cells, and podocytes resulting in progressive multi-organ failure and premature death and a milder form (1:1,390 males) that develops later in life associated with varying levels of α-Gal A activity.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Letters Source Type: research