Key result
Congenital deficiency of platelet cyclo-oxygenase resulted in impaired platelet release reaction, decreased aggregation with collagen and adrenalin, and absent aggregation with sodium arachidonate.
Case Report (n=2)
Congenital deficiency of platelet cyclo-oxygenase leads to impaired platelet aggregation and absent thromboxane A2 generation, highlighting the critical role of this pathway in platelet function.
Rare cyclo-oxygenase deficiency explains specific aggregation defects; leaves open diagnostic utility and prevalence in bleeding disorders.
Two cases of thrombocytopathia with congenital deficiency of platelet cyclo-oxygenase were investigated. The platelet release reaction was impaired. There was a marked decrease of aggregation with collagen and with adrenalin and a total absence of aggregation with sodium arachidonate. The platelet response to labile aggregation stimulating substance (LASS, mostly thromboxane A2) was normal. There was no biosynthesis of prostaglandin cyclic endoperoxides or of thromboxane A2 from arachidonic acid. Basal levels of platelet PGE1 were lowered although plasma levels were normal. Thrombin decreased the cyclic AMP content of patients' platelets and also that of control platelets pretreated with aspirin. The patients platelets showed no ultrastructural difference when compared with control platelets, except for a slight decrease of granule volume, but, in contrast to control platelets, thrombin (0.02 U/ml) did not provoke contraction of the patients' platelets.
No takes yet. Share an insight, caveat, or question.
Lagarde et al. (1978) conducted a case report in thrombocytopathia with congenital deficiency of platelet cyclo-oxygenase (n=2). Congenital deficiency of platelet cyclo-oxygenase vs. Control platelets was evaluated on Platelet release reaction and aggregation. Congenital deficiency of platelet cyclo-oxygenase resulted in impaired platelet release reaction, decreased aggregation with collagen and adrenalin, and absent aggregation with sodium arachidonate.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: