Abstract
ABSTRACT: The effect of red cell transfusion and polyethylene glycol-modified adenosine deaminase therapy on biochemical abnormalities, clinical status, and immunologic function in an adenosine deaminase-deficient child was investigated. After red cell transfusions, erythrocyte deoxyadenosine triphosphate (dATP) concentrations decreased about 95% and were closely related to adenosine deaminase activities; deoxyadenosine diphosphate concentrations decreased only approximately 30%. The evolution of dATP levels was also closely related to the improvement in clinical status of the patient. However, immune function was not restored. After polyethyelene glycol-modified adenosine deaminase therapy, the concentration of erythrocyte dATP decreased to undetectable levels correlated with an increase of T lymphocyte counts and an increase of lymphocyte responses to mitogens. Immune functions were restored only when dATP levels were below 15 μmol/L. It appears that red cell transfusion therapy is not sufficiently effective to reduce and maintain erythrocyte dATP levels at values compatible with normal immune function. On the contrary, polyethylene glycol-modified adenosine deaminase therapy is a suitable treatment to reduce dATP levels to near undetectable values, allowing the immune function to be restored. dATP measurement is a very useful tool for monitoring and evaluating the degree of efficiency of therapy in adenosine deaminase deficiency.
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Bory, C., Boulieu, R., Souillet, G. et al. Comparison of Red Cell Transfusion and Polyethylene Glycol-Modified Adenosine Deaminase Therapy in an Adenosine Deaminase-Deficient Child: Measurement of Erythrocyte Deoxyadenosine Triphosphate as a Useful Tool. Pediatr Res 28, 127–130 (1990). https://doi.org/10.1203/00006450-199008000-00010
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DOI: https://doi.org/10.1203/00006450-199008000-00010