[Thrombotic thrombocytopenic purpura: clinico-pathologic characteristics and therapy]

Thrombotic thrombocytopenic purpura (TTP) is disseminated form of thrombotic microangiopathy. Classic clinical presentation is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurologic symptoms, fever and impaired renal function. In the pathogenesis of TTP important role have changes in endothelial cells of small blood vessels, apoptosis, elevated endothelial derived proteins, anti-endothelial cell antibodies and platelet aggregating factors. In vast majority of patients with TTP deficient protease activity for von Willebrand factor (vWF), due to the presence of IgG antibodies is reported. Predisposing factors for development of TTP are infections, collagen vascular diseases, pregnancy, cancer, drugs, bone marrow transplantation, and genetic susceptability to TTP syndrome. Plasma exchange transfusions is the standard way of treatment. Additional treatment modalities with corticosteroids, cytotoxic drugs, antiplatelet drugs, intravenous immunoglobulins and splenectomy are applied.