Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency

Frank C. Schmalstieg, Gordon C. Mills, J. Arly Nelson, Linda T. May, Armond S. Goldman, Randall M. Golblum

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Abstract

A 10-month-old child with a profound deficiency of adenosine deaminase and severe combined immunodeficiency was treated for a period of 17 months with red cell and plasma transfusions containing normal amounts of the deficient enzyme. Following each transfusion, the plasma adenosine, red cell and lymphocyte ATP, urinary adenine, and urinary deoxyadenosine decreased transiently. During this period, the absolute blood lymphocyte count rose and a limited increased in the response of the lymphocytes to PHA-P was observed. Delayed hypersensitivity skin tests remained negative during the transfusion periods. A quantitative elevation of serum immunoglobulins occurred, but specific antibody formation was not elicited. In contrast to a previous report of successful therapy of ADA deficiency with red cell and plasma infusions, this patient responded poorly to enzyme replacement therapy. The difference may be related to a more profound enzyme deficiency in our patient.

Original languageEnglish (US)
Pages (from-to)597-603
Number of pages7
JournalThe Journal of Pediatrics
Volume93
Issue number4
DOIs
StatePublished - Oct 1978

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Schmalstieg, F. C., Mills, G. C., Arly Nelson, J., May, L. T., Goldman, A. S., & Golblum, R. M. (1978). Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency. The Journal of Pediatrics, 93(4), 597-603. https://doi.org/10.1016/S0022-3476(78)80894-4