INVITRO PLATELET ABNORMALITY IN ADENOSINE-DEAMINASE DEFICIENCY AND SEVERE COMBINED IMMUNODEFICIENCY

  • 1 January 1979
    • journal article
    • research article
    • Vol. 53  (3) , 465-471
Abstract
The platelets of an infant with severe combined immune deficiency and adenosine deaminase deficiency showed markedly diminished responses to ADP-induced aggregation in vitro. This abnormality was corrected by the addition of purified adenosine deaminase in vitro. Exogenous adenosine added to platelet-rich plasma caused markedly prolonged inhibition of ADP-induced aggregation. This was shown by isotopic studies to be due to slow clearance of adenosine and hence persistance of this nucleoside. Direct assay for adenosine deaminase in plasma and platelet lysates of the patient confirmed the very low activity of this enzyme. Raised c[cyclic]AMP levels were demonstrated in the platelets. The deranged adenosine metabolism and raised cAMP in the platelets of this child with severe combined immunodeficiency may explain the altered response to ADP. Despite the in vitro platelet aggregation abnormality, the patient had no clinical evidence of impaired hemostasis.

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