[Thrombocytopenic thrombotic purpura in adults. 10 cases (author's transl)]

Thrombocytopenic thrombotic purpura is a rare condition of controverted pathophysiology. The authors have observed and treated 10 cases between 1974 and 1979. The clinical features were uniform: haemolytic anaemia with schistocytosis (10/10), thrombopenia (10/10), fever (9/10), varying neurological disorders (9/10) which required assisted ventilation in 7 cases. Renal insufficiency was found in 7 patients, but associated with renal lesions only one and never interfered with the course of the disease. All patients received corticosteroids in high dosage and anti-platelet aggregation drugs. That treatment alone cured 3 patients. In addition, 5 patients underwent plasmapheresis, which seemed to be very effective in 2 cases. Five patients died, 3 of whom despite corticosteroids, anti-aggregants and plasmapheresis. Thus, none of these treatments seems to be effective in all cases, and prospective multicentre therapeutic trials are probably required.