This group of diseases is characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes.
Along with the well known routine laboratory parameters, measurement of factor H and metalloproteinases namely ADAMTS13 is recommended.
The pathogenetic link is the occlusion of microvessels by microthrombi. The resulting insufficient perfusion is responsible for the organ specific symptoms.
Nürnberger J et al. (2009) Eculizumab for atypical hemolytic-uremic syndrome.[^]
McCrae KR et al. (2001) Platelets: an update on diagnosis and management of thrombocytopenic disorders.[^]
Moake JL et al. (2002) Thrombotic microangiopathies.[^]
Shah NT et al. (2003) Controversies in differentiating thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.[^]