Table 3.

Complement serology at TMA episode

Patient	7	9	24	27	Reference range
TCA, classical pathway	84	16a	63	76	48–103 CH50/ml
TCA, alternative pathway	110	1a	68a	112	70–125 %
Complement C3	1.39	1.13	0.78a	1.0	0.9–1.8 g/l
Complement C4	0.64	0.28	0.39	0.38	0.15–0.55 g/l
Factor H ag	775	231a	333	366	250–880 mg/l
Complement factor I ag	111	59a	74	90	70–130%
Complement factor B ag	135	76	106	82	70–130%
Anti-factor H IgG autoantibody	25	n.a.	n.a.	18	<110 AU/ml
sC5b-9 (TCC)	n.a.	363a	384a	436a	110–252 ng/ml
C1q ag	100	56	69	115	60–180 mg/l
Anti-C1q IgG autoantibody	1	n.a.	n.a.	2	<52 U/ml
ADAMTS13 activity	84	n.a.	65	59	67–150 %

ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; ag, antigen; n.a., not available; TCA, total complement activity; TCC, terminal complement complex.

^aValues that do not lie within the reference range and are clinically relevant.