Introduction. The aim was to evaluate the effect of therapeutic
plasma exchange (TPE) and
eculizumab on hematological and renal survival in
atypical hemolytic uremic syndrome (aHUS), and additionally, to examine the reliability of discontinuation of
eculizumab treatment.Methods. This was an observational and retrospective study of 18 patients diagnosed with aHUS.Results. The median age of the study population was 30 (22-66) years. Four of 18 patients achieved hematological remission with the TPE alone. However, one patient died after three sessions of TPE.
Eculizumab was used in 13 patients and no death was observed. One year
after treatment, improved kidney function was observed in 2 of 3 (66%) patients for TPE and 5 of 9 (56%) patients for
Eculizumab. We discontinued
eculizumab treatment in 9 patients. One of the patients who had a C3 gene mutation experienced disease relapse after
Eculizumab discontinuation. None of the patients who had
drug associated aHUS developed disease relapse after
Eculizumab discontinuation.Conclusion.
Eculizumab treatment is a life-saving
therapy in aHUS. Treatment discontinuation may be considered at least six months after hematologic remission in patients who had stable renal function or no expectancy for renal survival. Moreover,
drug-associated cases seem to tend not to develop disease relapse in the long term.