Acquired storage pool deficiency with increased platelet-associated IgG. Report of five cases.
Acquired abnormalities of platelet aggregation have been reported with increasing frequency. We studied five patients (including two with systemic lupus erythematosus and one with compensated chronic idiopathic thrombocytopenic purpura) in whom platelet aggregation responses to collagen, epinephrine and ADP are impaired; in all cases, we found that levels of platelet-associated immunoglobulin G (IgG) were increased. In all five patients substances stored in platelet-dense granules (ATP, ADP, serotonin and calcium) were diminished. The content of the alpha-granule substance, beta-thromboglobulin, was also decreased in most cases, whereas the levels of two secretable acid hydrolase enzymes (beta-glucuronidase and beta-N-acetyl glucosaminidase) were within normal limits. These findings are similar to those observed in subtypes of congenital storage pool deficiency. However, in contrast to the congenital disorder, a membrane-bound (nonsecretable) acid phosphatase was also decreased in the patients with acquired storage pool deficiency. These findings suggest that impaired platelet aggregation on an acquired basis may, in some patients, be due to immune platelet damage resulting in a distinctive type of platelet storage pool deficiency.
D000249:Adenosine Monophosphate / Q000097:blood / Q000172:deficiency; D000255:Adenosine Triphosphate / Q000097:blood / Q000172:deficiency; D001792:Blood Platelets / Q000276:immunology; D002118:Calcium / Q000097:blood / Q000172:deficiency; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood*; D007074:Immunoglobulin G; D008180:Lupus Erythematosus, Systemic / Q000097:blood; D010974:Platelet Aggregation; D011696:Purpura, Thrombocytopenic / Q000097:blood; D012701:Serotonin / Q000097:blood / Q000172:deficiency; D013920:Thrombocythemia, Essential / Q000097:blood*; D001620:beta-Thromboglobulin / Q000172:deficiency
D007074:Immunoglobulin G; D001620:beta-Thromboglobulin; D012701:Serotonin; D000249:Adenosine Monophosphate; D000255:Adenosine Triphosphate; D002118:Calcium