Efficacy of plasma exchange in the therapy of recurrent pregnancy-associated atypical hemolytic-uremic syndrome

Background. Pregnancy-associated atypical hemolytic-uremic syndrome (aHUS) is characterized by an aggressive avalanche-like course and an extremely poor prognosis with fulminant thrombotic microangiopathy (TMA), whereas targeted therapy with a complement-blocking drug is always impossible in the first 24 hours. There are very few cases of recurrent pregnancy-associated aHUS in the modern literature; in this connection it is important to display the management of patients with the second disease episode that has developed during pregnancy. In the era of eculizumab, the role of plasma exchange in verified aHUS is becoming increasingly limited. Just the same, when eculizumab is not available, plasma exchange remains the only treatment option. Case report. The paper presents the experience in successfully treating two consecutive pregnancy-associated aHUS episodes manifested after delivery in one patient, by using only plasma exchange, which showed a favorable outcome, including complete recovery of renal function. Conclusion. Different types of TMA in pregnancy are fulminant and represent a life-threatening condition for the mother. In this condition, the most urgent issue is the timely initiation of appropriate therapy. The presented observation of two aHUS episodes demonstrates difficulties in choosing a treatment policy and in making a rapid differential diagnosis, even when there is only one episode of TMA in the history. The discussion lists the recommended treatment regimens, as well as the possibility of a favorable outcome with timely initiation of plasma therapy

plasma exchange, thrombotic microangiopathy, atypical hemolytic-uremic syndrome (a-HUS), pregnancy