Department of Nephrology , Hospital Universitario Dr Peset , Valencia , Spain.
Department of Pathology , Universitario Dr Peset Hospital , Valencia , Spain.
Clin Kidney J. 2015 Apr;8(2):232-6. doi: 10.1093/ckj/sfu144. Epub 2015 Jan 22.
Atypical haemolytic uraemic syndrome (aHUS) is a rare disease characterized by haemolytic microangiopathic anaemia, thrombocytopaenia and acute onset of renal failure, in the absence of Escherichia coli infection. Renal damage usually progresses to end-stage renal disease (ESRD), sometimes being accompanied by signs of extrarenal thrombotic microangiopathy (TMA). We report a case of full neurological and haematological recovery after eculizumab treatment in a patient with ESRD secondary to chronic aHUS refractory to plasmatherapy while she was under dialysis. It highlights the use of eculizumab for controlling extrarenal manifestations of aHUS in this population.
非典型溶血性尿毒症综合征 (aHUS) 是一种罕见的疾病,其特征为溶血性微血管病性贫血、血小板减少和急性肾衰竭,而无大肠埃希菌感染。肾脏损害通常进展为终末期肾病 (ESRD),有时伴有肾脏外血栓性微血管病 (TMA) 的迹象。我们报告了一例因慢性 aHUS 导致 ESRD 的患者,在透析期间,该患者对血浆疗法耐药,用依库珠单抗治疗后,其神经系统和血液学完全恢复。这突出了在该人群中使用依库珠单抗控制 aHUS 的肾脏外表现。