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Iloprost in the treatment of thrombotic microangiopathy: report of thirteen cases
A Sagripanti1, A Carpi, B Rosaia
1Clinical Medicine Institute, St Chiara University Hospital, Pisa, Italy.
Abstract:
Defective prostacyclin bioavailability seems to play a role in the pathogenesis of thrombotic microangiopathy, including thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Eight consecutive patients with a proven diagnosis of thrombotic microangiopathy were treated by Iloprost, a recently developed stable prostacyclin analogue; during follow-up, three of them relapsed and received further treatment. To our knowledge, this is the first report on a wide series of patients who received Iloprost for thrombotic microangiopathy. Soon after diagnosis, Iloprost was given by continuous intravenous infusion at a rate of 1.5-2 ng/kg/minute over 16-18 h/day for several days (mean 12 days; range 6-24) until the platelet count steadily increased. In addition, plasma exchange with fresh frozen plasma (average volume exchange 20-40 mL/kg for each session) was performed in 11 out of the 13 cases. No other antiplatelet agent was given. In all 13 cases, Iloprost administration coincided with achievement of remission. At present, all the patients are still maintaining remission. Our results indicate a useful role for Iloprost in the management of thrombotic microangiopathy.
Insights
Iloprost, a prostacyclin analogue, effectively treats thrombotic microangiopathy, including thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. This study shows Iloprost administration leads to remission in all treated patients, with sustained remission observed.
Area of Science:
- Hematology
- Pharmacology
Background:
- Thrombotic microangiopathy (TMA) pathogenesis involves defective prostacyclin bioavailability.
- Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are critical forms of TMA.
Observation:
- This study reports on thirteen patients diagnosed with TMA who were treated with Iloprost, a stable prostacyclin analogue.
- Iloprost was administered via continuous intravenous infusion (1.5-2 ng/kg/minute for 16-18 h/day, mean 12 days).
- Plasma exchange was used in conjunction with Iloprost in 11 cases; no other antiplatelet agents were administered.
Findings:
- Iloprost treatment, combined with plasma exchange in most cases, resulted in remission for all 13 patients.
- All patients maintained remission at the conclusion of the study.
- Three patients experienced relapses and required further treatment, indicating potential for recurrence.
Implications:
- Iloprost demonstrates a valuable role in managing thrombotic microangiopathy.
- This research highlights Iloprost as a potential therapeutic agent for TTP and HUS.
- Further investigation into long-term efficacy and optimal dosing of Iloprost in TMA is warranted.