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A 52-year old woman with congenital cyclooxygenase deficiency who had moderate bleeding tendency and severe generalized arteriosclerosis is reported.  Diagnosis based on the following data: secondary platelet aggregation induced by adrenaline, ADP, ristomycin, collagen, thrombin was absent; platelet completely failed to aggregate by arachidonic acid while aggregation induced by calcium ionophore A23187 was normal; no malondialdehyde formation could be detected in the platelet rich plasma /PRP/ in four different times /for exclu- sing any drug effects/. The abnormal adrenaline and ADP induced aggregation were not corrected when patient’s PRP was mixed in equal proportions with that of a normal subject ingesting aspirin.  Although our patient had been free from severe thrombotic episodes, expressed signs of generalized arteriosclerosis could be detected.  Until now only five cases of congenital deficiency of cyclo-oxygenase have been described /Malmsten et al., Weiss and Lages, Lagarde et al., Pareti et al./. However, in these patients no signs of arterial vascular diseases were mentioned.  The special importance of this new case comes from the fact that life-long deficiency of cyclooxygenase enzyme could not protect from progressive vascular disease which might prove again that chronic intake of large doses of aspirin cannot prevent arterial disorder.

Congenital Deficiency Of Cyclo-Oxygenase In A Woman With Generalized Arteriosclerosis