Hemolytiskt uremiskt syndrom och trombotisk trombocytopen purpura. Nya rön om EHEC, komplementmutationer och ADAMTS13.

Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with dysregulation of the alternative pathway of complement. TTP is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal and CNS manifestations. It has been associated with deficient activity of ADAMTS13, the von Willebrand factor cleaving protease. This review will focus on the classification, symptoms, pathology, pathogenesis, investigation, management and treatment of these conditions.