PURINE METABOLISM IN LYMPHOCYTES FROM PATIENTS WITH PRIMARY HYPOGAMMAGLOBULINEMIA

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  • 1 January 1978
    • journal article
    • research article
    • Vol. 31  (3) , 456-463
Abstract
The previous report of low levels of purine 5''-nucleotidase activity in peripheral blood mononuclear cells (lymphocytes and monocytes) from patients with non-familial adult onset variable primary hypogammaglobulinemia was confirmed and the observation extended to include patients with other types of primary immunodeficiency. Patients with sex-linked congenital hypogammaglobulinemia have values for mononuclear cell 5''-nucleotidase activity which are in the normal range; most cases of non-familial adult onset variable primary hypogammaglobulinemia have clearly subnormal values. The 3 patients with isolated Ig[immunoglobulin]A deficiency who were tested also had subnormal values. Evidence that the measured enzyme activity is in fact 5''-nucleotidase and independent of interfering phosphatase activities is presented. No significant or consistent alterations in the activities of the following enzymes were detected in mononuclear cells or erythrocytes: adenosine deaminase, purine nucleoside (inosine) phosphorylase, hypoxanthin phosphoribosyltransferase, adenine phosphoribosyltransferase or phosphoribosylpyrophosphate (PRPP) synthetase. The erythrocyte PRPP content and the mononuclear cell PRPP amidotransferase activity were normal in the small number of patients in which they were measured. The inter-relationship between some disorders of purine metabolism and the immunological deficiency syndromes was discussed.