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AHUS TTP PNH THrombotic microangiopathy

Eleni Gavriilaki, Xuan Yuan, Zhaohui Ye, Alexander J Ambinder, Satish P Shanbhag, Michael B Streiff, Thomas S Kickler, Alison R Moliterno, C John Sperati, Robert A Brodsky
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by excessive activation of the alternative pathway of complement (APC). Atypical HUS is frequently a diagnosis of exclusion. Differentiating aHUS from other TMAs, especially thrombotic thrombocytopenic purpura (TTP), is difficult due to overlapping clinical manifestations. We sought to develop a novel assay to distinguish aHUS from other TMAs based on the hypothesis that paroxysmal nocturnal hemoglobinuria cells are more sensitive to APC-activated serum due to deficiency of glycosylphosphatidylinositol- anchored complement regulatory proteins (GPI-AP)...
June 4, 2015: Blood
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