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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 persistence of this nucleoside. Direct assay for adenosine deaminiase in plasma and platelet lysates of the patient confirmed the very low activity of this enzyme. Raised cAMP levels were demonstrated in his 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.
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
February 1980,
Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
April 1975,
The New England journal of medicine,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
June 1978,
British journal of haematology,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
January 1980,
Advances in enzymology and related areas of molecular biology,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
May 1977,
Life sciences,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
November 1973,
Lancet (London, England),
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
June 1973,
Lancet (London, England),
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
March 2012,
JPMA. The Journal of the Pakistan Medical Association,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
October 1979,
The Tohoku journal of experimental medicine,
C H Lee, and
S P Evans, and
M C Rozenberg, and
A S Bagnara, and
J B Ziegler, and
M B Van der Weyden
May 1982,
Anales espanoles de pediatria,
