Acquired thrombotic thrombocyto-penic purpura is a rare disease, which is almost always fatal within a few days, in the absence of treatment. It is characterised by a combination of wide-ranging organ damage due to multiple thromboses, thrombo-cytopenia related to aggregation of platelets within thrombi, and haemolytic anaemia. The condition is linked to an increase in levels of von Willebrand factor, a procoagulant protein (1,2). Treatment of an episode is based on plasma exchange, usually for several days. Relapses are frequent, often after a relatively short interval, but they sometimes occur after several years (1).
展开▼