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Enzyme replacement therapy in a patient with Fabry disease and the development of IgE antibodies against agalsidase beta but not agalsidase alpha.
J Inherit Metab Dis. 2010 Dec; 33 Suppl 3:S249-52.JI

Abstract

Fabry disease is an X-linked inherited lysosomal storage disorder caused by an inborn deficiency of the enzyme α-galactosidase A. Enzyme replacement therapy (ERT) with agalsidase alpha or beta isozymes is an effective treatment. Cross-reactivity of immunoglobulin G (IgG) antibodies with agalsidase alpha and beta has been reported, but no such reaction has been recorded for IgE antibodies. We present the case of a patient with Fabry disease who developed antiagalsidase beta IgE antibodies without cross-reactivity to agalsidase alpha. A 17-year-old boy with Fabry disease had suffered from severe atopic dermatitis since infancy, and he complained for several years of peripheral pain during the summer months and when exercising. Fabry disease was confirmed by family history and a positive enzyme test, and ERT was commenced. Following infusion of agalsidase beta (1.0 mg/kg), the patient complained of a high temperature in his hands and feet, and purulent eczema developed. The infusion dose was reduced to 0.2 mg/kg, but the hyperthermia did not change, although its duration decreased. After three infusions, eosinophilia developed (9.4%; 573 cells/μl blood) and remained unresolved after four infusions with agalsidase beta. Treatment with this enzyme was discontinued, and agalsidase alpha (0.2 mg/kg) started. This produced immediate resolution of the eosinophilia, which has been maintained during follow-up. In conclusion, this patient developed IgE antibodies against agalsidase beta, which demonstrated no cross-reactivity to agalsidase alpha. These findings emphasize the importance of analyzing IgE antibodies against both enzymes when patients exhibit severe infusion-related events.

Authors+Show Affiliations

Department of Pediatrics, Osaka City University, Graduate School of Medicine, Osaka, Japan. akemi-chan@med.osaka-cu.ac.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

20567910

Citation

Tanaka, Akemi, et al. "Enzyme Replacement Therapy in a Patient With Fabry Disease and the Development of IgE Antibodies Against Agalsidase Beta but Not Agalsidase Alpha." Journal of Inherited Metabolic Disease, vol. 33 Suppl 3, 2010, pp. S249-52.
Tanaka A, Takeda T, Hoshina T, et al. Enzyme replacement therapy in a patient with Fabry disease and the development of IgE antibodies against agalsidase beta but not agalsidase alpha. J Inherit Metab Dis. 2010;33 Suppl 3:S249-52.
Tanaka, A., Takeda, T., Hoshina, T., Fukai, K., & Yamano, T. (2010). Enzyme replacement therapy in a patient with Fabry disease and the development of IgE antibodies against agalsidase beta but not agalsidase alpha. Journal of Inherited Metabolic Disease, 33 Suppl 3, S249-52. https://doi.org/10.1007/s10545-010-9136-0
Tanaka A, et al. Enzyme Replacement Therapy in a Patient With Fabry Disease and the Development of IgE Antibodies Against Agalsidase Beta but Not Agalsidase Alpha. J Inherit Metab Dis. 2010;33 Suppl 3:S249-52. PubMed PMID: 20567910.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Enzyme replacement therapy in a patient with Fabry disease and the development of IgE antibodies against agalsidase beta but not agalsidase alpha. AU - Tanaka,Akemi, AU - Takeda,Taisuke, AU - Hoshina,Takao, AU - Fukai,Kazuyoshi, AU - Yamano,Tsunekazu, Y1 - 2010/06/22/ PY - 2010/03/04/received PY - 2010/05/18/accepted PY - 2010/05/14/revised PY - 2010/6/23/entrez PY - 2010/6/23/pubmed PY - 2014/4/9/medline SP - S249 EP - 52 JF - Journal of inherited metabolic disease JO - J Inherit Metab Dis VL - 33 Suppl 3 N2 - Fabry disease is an X-linked inherited lysosomal storage disorder caused by an inborn deficiency of the enzyme α-galactosidase A. Enzyme replacement therapy (ERT) with agalsidase alpha or beta isozymes is an effective treatment. Cross-reactivity of immunoglobulin G (IgG) antibodies with agalsidase alpha and beta has been reported, but no such reaction has been recorded for IgE antibodies. We present the case of a patient with Fabry disease who developed antiagalsidase beta IgE antibodies without cross-reactivity to agalsidase alpha. A 17-year-old boy with Fabry disease had suffered from severe atopic dermatitis since infancy, and he complained for several years of peripheral pain during the summer months and when exercising. Fabry disease was confirmed by family history and a positive enzyme test, and ERT was commenced. Following infusion of agalsidase beta (1.0 mg/kg), the patient complained of a high temperature in his hands and feet, and purulent eczema developed. The infusion dose was reduced to 0.2 mg/kg, but the hyperthermia did not change, although its duration decreased. After three infusions, eosinophilia developed (9.4%; 573 cells/μl blood) and remained unresolved after four infusions with agalsidase beta. Treatment with this enzyme was discontinued, and agalsidase alpha (0.2 mg/kg) started. This produced immediate resolution of the eosinophilia, which has been maintained during follow-up. In conclusion, this patient developed IgE antibodies against agalsidase beta, which demonstrated no cross-reactivity to agalsidase alpha. These findings emphasize the importance of analyzing IgE antibodies against both enzymes when patients exhibit severe infusion-related events. SN - 1573-2665 UR - https://www.unboundmedicine.com/medline/citation/20567910/Enzyme_replacement_therapy_in_a_patient_with_Fabry_disease_and_the_development_of_IgE_antibodies_against_agalsidase_beta_but_not_agalsidase_alpha_ L2 - https://doi.org/10.1007/s10545-010-9136-0 DB - PRIME DP - Unbound Medicine ER -