Deficiency of plasma prostacyclin or PGI2 regenerating ability in sickle cell anaemia.
We have demonstrated that patients with sickle cell disease greater than 2 years of age have a marked decrease in their plasma PGI2 or prostacyclin regenerating ability (0.05 +/- 0.08 ng/mg of vascular tissue) when compared to normal controls (0.44 +/- 0.12). This abnormality was not present in other chronic haemolytic states, and was not related to concomitant hyposplenism. Normal activity was observed in patients with sickle cell disease following a period of prophylactic red cell transfusions (0.32 +/- 0.13). Since vascular prostacyclin is antiaggregatory and antithrombotic, the deficiency in the prostacyclin regenerating ability of patients with sickle cell disease may play a pathogenic role in the development of the microvascular and thrombotic complications seen in this disorder.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000628:therapy; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D011464:Epoprostenol / Q000096:biosynthesis* / Q000097:blood; D017707:Erythrocyte Transfusion; D006801:Humans; D007223:Infant; D011453:Prostaglandins / Q000096:biosynthesis*
D011453:Prostaglandins; D011464:Epoprostenol