[Thrombotic thrombocytopenic purpura (Moschowitz syndrome). A clinical case and review of the literature]. / Porpora trombotica trombocitopenica (sindrome di Moschowitz). Caso clinico e revisione della letteratura.

ABSTRACT

A case of young woman affected by relapsing Thrombotic Thrombocytopenic Purpura (TTP), probably beginning in September 1986 and relapsing after 6 years, in June 1992, is described. After a first Complete Remission due to Plasma Exchange and Antiplatelet Agents, the patient relapsed during maintenance therapy with Ticlopidine and, over 1 month, developed two relapses which were resistant to traditional therapy. Showing resistance even to Iloprost, the patient had a Complete Remission only after a 2nd IV bolus of Vincristine according to Gutterman's scheme (modified), with prolonged therapeutic wellbeing. Many authors say that Plasma Exchange and Vincristine therapy must first be used in TTP and tried in refractory TTP. This one experience of ours should confirm that. From this TTP case, the authors begin a wide review of the literature, examining history, etiopathogenetic and therapeutic debate, possible related diseases, differential diagnosis, evolution, prognosis and future developments of this mysterious and rare illness.