Sengul Samanci Nilay, Ayer Mesut, Ergen Abdulkadir, Ozturk Savas
Department of Internal Medicine, Haseki Training and Research Hospital, Istanbul, Turkey.
Department of Hematology, Haseki Training and Research Hospital, Istanbul, Turkey.
Transfus Apher Sci. 2015 Jun;52(3):314-6. doi: 10.1016/j.transci.2015.01.006. Epub 2015 Jan 12.
Atypical hemolytic uremic syndrome is a rare thrombotic microangiopathy caused by chronic defective regulation of the complement activation. This activation results in systemic endothelial damage leading to renal failure. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS and has recently came out as a therapeutic option for aHUS. Here we present a case showing that first-line eculizumab treatment successfully prevents the induction of the terminal complement cascade and blocked the progression of thrombotic microangiopathy in aHUS.
非典型溶血性尿毒症综合征是一种罕见的血栓性微血管病，由补体激活的慢性调节缺陷引起。这种激活导致全身内皮损伤，进而导致肾衰竭。依库珠单抗是一种抗C5抗体，对限制非典型溶血性尿毒症综合征患者的补体激活有效，最近已成为非典型溶血性尿毒症综合征的一种治疗选择。在此，我们报告一例病例，显示一线依库珠单抗治疗成功预防了终末补体级联反应的诱导，并阻断了非典型溶血性尿毒症综合征中血栓性微血管病的进展。
