Deficiency of Plasma Prostacyclin or PGI2 Regenerating Ability in Sickle Cell Anaemia

Abstract

Patients with sickle cell disease > 2 yr of age have a marked decrease in their plasma PG[prostaglandin]I2 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 hemolytic 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.