Abnormal Platelet Aggregation in Severe Combined Immunodeficiency Disease with Adenosine Deaminase Deficiency

The platelets from a boy with adenosine deaminase deficiency‐severe combined immunodeficiency disease (ADA‐SCID) showed markedly subnormal aggregation in response to collagen and ADP. In contrast with normal platelets, ATP, AMP and adenosine had little effect in inhibiting ADP‐induced aggregation of ADA‐SCID platelets. These observations suggest that altered adenosine metabolism exists in ADA‐SCID platelets.