Summary box plots for the levels of complement activation products in healthy controls (N=40) and in patients with TTP (N=48), aHUS (N=41), or GC-aHUS (N=9) measured with ELISA. (A) C3a [ng/mL: control, 139.8 (31–473.8); TTP, 195.9 (62–404.4); aHUS, 221.3 (80.7–791.8); GC-aHUS, 266.4 (115.7–483.8); P=0.005]. (B) Factor Bb [ng/mL: control, 499 (110–2,072); TTP, 760 (1–16,557); aHUS, 1,200 (270–11,797); GC-aHUS, 1,380 (52–2490); P<0.001]. (C) C5a [ng/mL: control, 11.4 (2.7–69.1); TTP, 20.1 (2.7–68.6); aHUS, 19.9 (5.4–66.1); GC-aHUS, 22.7 (8.7 –37.4); P=0.001]. (D) C5b-9 [ng/mL: control, 155 (58–459); TTP, 292 (85–939); aHUS, 317 (82–1,415); GC-aHUS, 340 (183–515); P<0.001]. All the data represent the medians (ranges). The P-values were calculated using the Kruskal-Wallis test.

Abbreviations: aHUS, atypical hemolytic uremic syndrome; GC-aHUS, genetically confirmed aHUS; NS, not significant; TTP, thrombotic thrombocytopenic purpura.