Back to Search Start Over

Management of hypersensitivity reactions to enzyme replacement therapy in children with lysosomal storage diseases.

Authors :
Turgay Yagmur I
Unal Uzun O
Kucukcongar Yavas A
Kulhas Celik I
Toyran M
Gunduz M
Civelek E
Dibek Misirlioglu E
Source :
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology [Ann Allergy Asthma Immunol] 2020 Oct; Vol. 125 (4), pp. 460-467. Date of Electronic Publication: 2020 Jul 17.
Publication Year :
2020

Abstract

Background: Intravenous recombinant enzyme replacement therapy (ERT) is currently available for 8 lysosomal diseases. Hypersensitivity reactions (HSRs) may be observed during this long-term treatment.<br />Objective: To evaluate the frequency and clinical treatment features of ERT HSRs and the management of desensitizations in children.<br />Methods: Medical records were reviewed retrospectively for patients who received ERT. Those who had experienced HSRs to ERT were included in the study. The demographic characteristics of the patients, culprit enzyme, signs and symptoms, diagnostic tests, management of the reaction, and the protocol employed for the maintenance of ERT were recorded.<br />Results: During the study period, 54 patients received ERT in our institution. A total of 11 patients (20.4%) experienced HSR to ERT. All reactions were of immediate type. The most common symptoms were cutaneous manifestations. A total of 9 patients experienced urticaria, and 2 had anaphylaxis as initial reaction. Patients who had isolated cutaneous symptoms continued their treatments with antihistamines, corticosteroid premedication, slower infusion rate or both. Patients who had recurrent urticaria with these modalities or those who had anaphylaxis continued their ERT with desensitization (n = 8). A total of 3 patients required revisions in desensitization protocols because of recurrent anaphylaxis.<br />Conclusion: The reactions that develop during this long-term treatment may be treated by premedication-prolonged infusion, but in some patients, desensitization protocols are necessary for the continuation of therapy. Revisions in desensitization protocols may be required.<br /> (Copyright © 2020 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Details

Language :
English
ISSN :
1534-4436
Volume :
125
Issue :
4
Database :
MEDLINE
Journal :
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology
Publication Type :
Academic Journal
Accession number :
32687987
Full Text :
https://doi.org/10.1016/j.anai.2020.07.010