(1) Baskent University, Ankara, Turkey
(2) Adana Numune Training and Research Hospital, Adana, Turkey
* Corresponding author Email:
Atypical haemolytic uremic syndrome is a result of a spectrum of diseases. Disorders of complement regulation are the most important reasons in the aetiology. It is associated with defective regulation of the alternative complement pathway in over 50% of cases. Clinical abnormalities are related with the presence of thrombotic microangiopathy. Patients with atypical haemolytic uremic syndrome have a poor prognosis with a high mortality and morbidity in the acute phase of the disease and progression to end-stage renal disease in 50% of the cases. Various extra renal complications due to systemic thrombotic microangiopathy may occur in HUS, including neurological, pancreatic and cardiac involvement. Eculizumab is a humanized monoclonal anti-C5 antibody. It blocks the alternative complement pathway at the level of proinflammatory C5a and lytic C5b-9 complex generation. We discuss haemolytic uremic syndrome and treatment options.
Related to increase of experiences, eculizumab therapy may be the first-line treatment. We do not know optimal duration of eculizumab therapy. We do not know also in which patient a severe relapse could be developed. At this moment we can suggest that in both cases eculizumab is life-saving and enhancing the quality of life.