Pregnancy-Associated Atypical Hemolytic Uremic Syndrome (Ahus), Treated With Eculizumab
BLOOD(2014)
Abstract
Introduction:aHUS is a life-threatening, chronic, progressive disease of complement mediated thrombotic microangiopathy (TMA), commonly associatedwith pregnancy, 10-20% of all cases (P-aHUS), that usually occurs in late pregnancy and postpartum, primiparous women. Plasma exchange (PEX) may transiently maintain hematologic parameters, but does not treat the underlying systemic disease. Clinical studies have shown excellent effectiveness of eculizumab (Soliris®), the only complement inhibitor, used in PNH treatment. In 2011, eculizumab was approved for the treatment of aHUS. We report the 1st case of aHUS treated with eculizumab, in Greece, which was pregnancy related.
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Key words
eculizumab,ahus,syndrome,pregnancy-associated
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