Fig. 1. Diagnostic algorithm and therapeutic options for posttransplant TMA. If TMA of unknown cause occurs after kidney transplantation or recurrent TMA is observed, ADAMTS13 and Shiga toxin assay tests need to be performed to differentiate aHUS from TTP and STEC-HUS, along with genetic testing, and consider early initiation of eculizumab. If secondary TMA is suspected, trigger management should be performed first, but if there is no response to treatment for the secondary cause or if plasmapheresis-dependent patterns are observed, the diagnosis and treatment of aHUS should be considered. TMA, thrombotic microangiopathy; PPx, plasma exchange; ABMR, antibody-mediated rejection; RTX, rituximab; IVIG, intravenous immune globulin; STEC, Shiga toxin-producing Escherichia coli; MCP, membrane cofactor protein; CFH, complement factor H; CFI, complement factor I; CFB, complement factor B; THBD, thrombomodulin; TTP, thrombotic thrombocytopenic purpura; HUS, hemolytic uremic syndrome; aHUS, atypical HUS.
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