Table 3

Comparison of pretreatment complement biomarkers obtained from 19 patients with clinically diagnosed aHUS vs a cohort of 38 patients with ADAMTS13-deficient TTP

Clinical diagnosisComplement biomarkers (ng/mL)
Factor Bb (244.3-960.8)C4d (278.5-1845.9)C5b-9 (33.9-238.2)C5a (18.6-47.9)C3a (6.9-242.3)
Acquired TTP* (n = 38) 2153 (343-5448) 3534 (458-7450) 585 (210-1924) 75 (29-210) 777 (128-4782) 
aHUS* (n = 19) 7386 (603-30 610) 2914 (1394-15 210) 1098 (422-4840) 115 (55-280) 1237 (79-13 730) 
P value .063 .706 < .0001 .004 .031 
Clinical diagnosisComplement biomarkers (ng/mL)
Factor Bb (244.3-960.8)C4d (278.5-1845.9)C5b-9 (33.9-238.2)C5a (18.6-47.9)C3a (6.9-242.3)
Acquired TTP* (n = 38) 2153 (343-5448) 3534 (458-7450) 585 (210-1924) 75 (29-210) 777 (128-4782) 
aHUS* (n = 19) 7386 (603-30 610) 2914 (1394-15 210) 1098 (422-4840) 115 (55-280) 1237 (79-13 730) 
P value .063 .706 < .0001 .004 .031 

Compared with the acquired TTP patients, patients clinically diagnosed aHUS tended to have significantly higher C5b-9 and C5a levels. P values reflect comparisons of complement biomarkers between diagnosis groups using Wilcoxon rank sum tests. Bolded text signifies the values that were statistically significant at the P < .05 level.

*

For the acquired TTP patients, the mean data for each complement biomarker are shown; whereas for the smaller aHUS cohort, the median biomarker data are presented.

Compared with the 19 clinically diagnosed aHUS patients. The Mann-Whitney independent U test was used for the Bb and C4d comparisons, whereas a generalized linear model was used to compare C5b-9, C5a, and C3a, using age, sex, and race as covariables.

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