Intravenous prostacyclin (as epoprostenol) infusion in thrombotic thrombocytopenic purpura. Four case reports and review of the literature

• Published in: Haematologica (Roma) 1994-01, Vol.79 (5), p.429-437
• Main Authors: Bobbio-Pallavicini, E, Porta, C, Tacconi, F, Gugliotta, L, Centurioni, R, Vianelli, N, Billio, A, Ascari, E
• Format: Article
• Language: English
• ISSN: 0390-6078

The enhanced platelet aggregation which is observed in TTP, was suggested to be due to an imbalance between unknown agents insulting endothelial wall and defense factors, such as prostacyclin (PGI sub(2)). Several reports suggested an aberration of PGI sub(2) activity as a critical step in the pathogenesis of TTP. Therefore, PGI sub(2) was proposed as an alternative treatment for TTP patients. We report the results obtained with increasing doses (from 2 ng/Kg/min to 10 ng/Kg/min in 5 days) of PGI sub(2) - as epoprostenol - in 4 TTP patients from the retrospective series of the Italian Cooperative Group who were considered resistant to conventional plasma-exchange (PE)-based treatments. Despite PGI sub(2) infusion, 2 patients died, while the extant 2 achieved stable complete remission. Notably, the only patient whose PE was administered with adequate frequency and for an adequate period of time, and thus the only unquestionably PE-resistant patient, was also resistant to PGI sub(2) infusion. Major side-effects were few and observed at the highest doses. In our experience and from the analysis of the literature, which, as far as we know, includes only 23 patients treated with PGI sub(2)-like substances, the role of PGI sub(2) in the treatment of TTP appears to be modest. Maybe the identification of subgroups of TTP patients exhibiting some defects in PGI sub(2) metabolism, together with the use of more manageable PGI sub(2) analogs, such as iloprost, could revive interest in these molecules in the future.