Atypical hemolytic-uremic syndrome due to complement factor I mutation

Figure 1 Finding of renal biopsy showed. A: A glomerulus with extensive fibrin thrombi involvement, obliterating the capillary loops (Periodic Acid-Schiff stain, original magnification × 400); B: A glomerulus with an afferent arteriole with extensive red, fuchsinophilic material (fibrin) involvement (Masson trichrome stain, original magnification × 400); C: A small arteriole with luminal fibrin staining red (fuchsinophilic) (Masson trichrome stain, original magnification × 400).

Figure 2 Serum creatinine levels did not improve despite plasma exchange, and hemodialysis was initiated. Lactate dehydrogenase levels improved with plasma exchange, whereas the platelets showed transient improvement under the plasma exchange treatment. Treatment with eculizumab induced a persistent improvement in platelet count. Continued eculizumab treatment resulted in late renal benefit, with dialysis withdrawn in June 2015. PLEX: Plasma exchange; Scr: Serum creatinine; LDH: Lactate dehydrogenase.