LONG-TERM PLASMA INFUSIONS IN A PATIENT WITH ATAXIA-TELANGIECTASIA AND DEFICIENT IGA AND IGE

Author:

Ammann A. J.1,Good R. A.1,Bier D.1,Fudenberg H. H.1

Affiliation:

1. Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota, and the Departments of Medicine and Pediatrics, University of California, San Francisco

Abstract

A patient with ataxia-telangiectasia and deficient IgA and IgE has been treated with long-term plasma infusions from a single donor over a 3-year period. No significant deleterious effects could be demonstrated. Despite the presence of anti-IgA antibodies, plasma infusions did not result in transfusion reactions. The IgA levels achieved following plasma infusion were similar to that of the donor. IgE could be detected in the skin of thee patient 5 days following plasma infusion, but no IgA was demonstrated in the saliva. Some evidence of a beneficial effect is suggested a significant control of recurrent sinopulmonary infection in a patient with deficient IgA, IgE, and abnormal cellular immunity. Currently plasma infusion is the only readily available method of replacing adequate amounts of deficient IgA and IgE. Bone marrow transplantation was considered in this patient, but lack of a suitable donor has made this form of treatment temporarily unavailable.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology, and Child Health

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