iloprost and Purpura--Thrombotic-Thrombocytopenic

Thrombotic microangiopathy, including the two related syndromes thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome, is a rare and severe multisystem disorder, due to widespread deposition of intravascular microthrombi consisting mainly of platelets, with subsequent consumption thrombocytopenia, microangiopathic hemolytic anemia, renal abnormalities, and neurologic disturbances. The epidemic, verotoxin-induced hemolytic-uremic syndrome, typically associated with prodromal diarrhea, mainly affects young children in small outbreaks. By contrast, idiopathic thrombotic microangiopathy generally affects adults in a sporadic form; it has a more devastating course and a less favourable outcome. Over 90% of the reported cases in the adult, when untreated, have progressed to death within three months of diagnosis. Since the introduction of plasma exchange, a dramatic change in the prognosis of the disease has taken place, although the mortality rate still remains considerable. Indeed, improved survival is the most striking feature of adult thrombotic microangiopathy compared to some decades ago. In the present article we will focus on the evolving concepts able to exert a considerable impact in the management of the adult idiopathic form of thrombotic microangiopathy.

Topics: Adult; Hemolytic-Uremic Syndrome; Humans; Iloprost; Multiple Organ Failure; Plasma Exchange; Platelet Aggregation; Platelet Aggregation Inhibitors; Purpura, Thrombotic Thrombocytopenic; Thrombosis

Prostacyclin has been suggested as a useful agent for patients with thromobotic thrombocytopenic pupura (TTP) refractory to plasma-exchange. We report our unsuccessful experience with iloprost in a patient with TTP resistant to plasma-exghange, vincristine and high dose immunoglobulins.

Topics: Adult; Drug Resistance; Epoprostenol; Female; Humans; Iloprost; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Treatment Failure

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.

Topics: Adolescent; Adult; Drug Administration Schedule; Female; Hemolytic-Uremic Syndrome; Humans; Iloprost; Male; Middle Aged; Plasma Exchange; Platelet Aggregation Inhibitors; Purpura, Thrombotic Thrombocytopenic