In vitro platelet abnormality in adenosine deaminase deficiency and severe combined immunodeficiency.

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 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.

journal_name

Blood

journal_title

Blood

authors

Lee CH,Evans SP,Rozenberg MC,Bagnara AS,Ziegler JB,Van der Weyden MB

subject

Has Abstract

pub_date

1979-03-01 00:00:00

pages

465-71

issue

3

eissn

0006-4971

issn

1528-0020

journal_volume

53

pub_type

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