Abstract

ALMAGRO VAZQUEZ, Delfina. Some considerations about the thrombocytopenic thrombotic purpura  . Rev Cubana Hematol Inmunol Hemoter [online]. 2007, vol.23, n.1, pp. 0-0. ISSN 1561-2996.

The thrombocytopenic thrombotic purpura is a disease characterized by microangiopathic hemolytic anemia, thombocytopenia , fluctuating neurological manifestations, and renal disorders. The occlusion of arterioles and capillars by microthrombi mainly composed of platelets is typical of this disorder and a consequence of the presence of large von Willebrand factor multimers, due presumptively to the reduction of the activity of the enzyme ADAMTS13 that is in charge of splitting these multimers.The early diagnosis of TTP allows a fast and efficient treatment of this disorder, a decisive element to prevent the fatal evolution of these patients. The presence of anemia and thrombocytopenia nonexplained by other pathological processes makes us suspect the diagnosis. The plasmatic turnover is the most effective therapeutics, and it should be considered the elective treatment of TTP. The use of immunosuppressors associated with the plasmatic turnover has been recommended in some cases. Nowadays, rituximab is regarded as an additional treatment of TTP

Keywords : Thrombocytopenic thrombotic purpura; microangiopathic hemolytic anemia; thrombocytopenia; multimers; von Willebrand factor; ADAMST 13.