Juraj Mariš, Diana Martinková
Atypical haemolytic uraemic syndrome is thrombotic microangiopathy (TMA) characterized by non-imune hemolytic
anemia, thrombocytopaenia and by impairment of at least one organ, the most frequently kidneys, nervous and cardiovascular
system. Pathophysiologicaly, the main role plays overactivation of alternative complement pathway, which
causes endothelial damage, microthrombosis and activation of inflamation. Up to 60% of patients have inherited mutation
of complement genes, rarely anti-complement factor H antibodies. Activation of complement can be caused by
infection, malignity, surgery and various medication. In differential diagnosis, other the most frequent TMAs as typical
HUS, TTP and DIC have to be excluded. For aHUS, mild or moderate grade of thrombocytopenia, serious renal impairment
and activity od ADAMTS-13 > 10% is more typical. According to type of complement mutation we are able to
establish the diagnosis, determine the prognosis and risk of reccurence, suggest suitable therapeutic strategy and predict
response to it. Nowadays, eculizumab by effective complement blockade allows termination of TMA and restoration
of organs function.