Premium
Deficiency of Plasma Prostacyclin or PGI 2 Regenerating Ability in Sickle Cell Anaemia
Author(s) -
Stuart Marie J.,
Sills Richard H.
Publication year - 1981
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1981.tb02751.x
Subject(s) - prostacyclin , medicine , antithrombotic , sickle cell anemia , endocrinology , disease , immunology
S ummary . We have demonstrated that patients with sickle cell disease > 2 years of age have a marked decrease in their plasma PGI 2 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.