ABSENCE OF LYMPHOCYTE ECTO-5'-NUCLEOTIDASE IN INFANTS WITH RETICULOENDOTHELIOSIS AND EOSINOPHILIA (OMENNS SYNDROME)

  • 1 January 1984
    • journal article
    • research article
    • Vol. 63  (6) , 1475-1480
Abstract
Lymphocytes from 3 infants with reticuloendotheliosis and eosinophilia (Omenn''s syndrome) and immunodeficiency were assayed for 5''-nucleotidase activity. B and T lymphocytes from all 3 patients were totally deficient in ecto-5''-nucleotidase activity, but had normal levels of cytoplasmic 5''-nucleotidase. In contrast, cultured lymphocytes expressed normal ectoplasmic and cytoplasmic activities, suggesting that the lymphocyte-restricted enzyme deficiency was not likely a primary genetic defect. The deficiency of lymphocyte ecto-5''-nucleotidase was not associated with any abnormality of deoxynucleoside metabolism. The absence of lymphocyte ecto-5''-nucleotidase may be a characteristic feature of this syndrome and may help to distinguish this disease from others with similar manifestations.

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