Table 3 Complement profile in... | American Society of Hematology

Table 3

Complement profile in eculizumab-treated aHUS patients

Case	Mutation or anti-CFH Ab	Disease phase treatment	Clinical parameters*				Complement parameters
Case	Mutation or anti-CFH Ab	Disease phase treatment	Platelets (150-400 × 10³/μL)	LDH (266-500 IU/L)	Hemoglobin (14-18 g/dL)	Serum creatinine (0.55-1.25 mg/dL)	Serum C3 (83-180 mg/dL)†	Plasma SC5b-9 (127-400 Eq/mL)†	Serum CH50 (79-187 U Eq/mL)†	C5b-9 deposits‡ (% of control)
1	CFHR1/CFH	Acute (pre-Ecu)	111 000	2000	5.1	6	79	329	227	309§
1	Hybrid gene	9 d post-Ecu 1200 mg	318 000	741	12.6	1.7	91	533	5	71
2	CFB -L433S	Acute (pre-Ecu)	121 000	2011	5.5	27	77	156	100	1019§
2	CFB -L433S	14 d post-Ecu 1200 mg	280 000	357	12.9	1.22	65	967	11	106
3	No mutations	Acute (pre-Ecu)	19 000	2373	9.2	1.9	50	411	142	478§
3	No anti-CFH Ab	21 d post-Ecu 300 mg	564 000	n.d.	12.5	0.6	76	116	46	105
4	No mutations	Acute (pre-Ecu)	107 000	768	9.6	15.2	78	556	45	439§
4	No anti-CFH Ab	14 d post-Ecu 1200 mg	161 000	342	11.7	4.77	88	260	4	59
5	CFH-S1191L	Remission (pre-Ecu)	237 000	576	10.9	8.91	93	121	72	298§
		8 d post-Ecu 300 mg	67 000	560	13.5	0.52	103	195	3	152§
		12 d post-Ecu 600 mg	119 000	588	13.6	0.59	95	214	3	43
		11 d post-Ecu 600 mg	225 000	493	12.6	0.5	n.d.	167	6	76
6	C3-K633R	Acute (pre-Ecu)	39 000	5000	7.7	1.48	123	392	93	342§
		6 d post-Ecu 600 mg	159 000	854	8.5	4.29	126	231	1	119
		6 d post-Ecu 900 mg	232 000	473	11.5	1.5	n.d.	566	11	82
7	CFH-R1210C	Acute (pre-Ecu)	46 000	1962	7	5.7	79	421	118	474§
		14 d post-Ecu 1200 mg	277 000	336	11.9	1.4	89	505	10	102
		21 d post-Ecu 1200 mg	307 000	264	12.2	1.2	83	231	32	75
8	CFI-R187Q	Acute (pre-Ecu)	94 000	869	7.2	4.5	99	534	n.d.	177§
		14 d post-Ecu 1200 mg	212 000	401	10.1	2	89	435	6	24
		1 mo post-Ecu 1200 mg	185 000	434	11.6	1.93	85	298	22	92

Case	Mutation or anti-CFH Ab	Disease phase treatment	Clinical parameters*				Complement parameters
Case	Mutation or anti-CFH Ab	Disease phase treatment	Platelets (150-400 × 10³/μL)	LDH (266-500 IU/L)	Hemoglobin (14-18 g/dL)	Serum creatinine (0.55-1.25 mg/dL)	Serum C3 (83-180 mg/dL)†	Plasma SC5b-9 (127-400 Eq/mL)†	Serum CH50 (79-187 U Eq/mL)†	C5b-9 deposits‡ (% of control)
1	CFHR1/CFH	Acute (pre-Ecu)	111 000	2000	5.1	6	79	329	227	309§
1	Hybrid gene	9 d post-Ecu 1200 mg	318 000	741	12.6	1.7	91	533	5	71
2	CFB -L433S	Acute (pre-Ecu)	121 000	2011	5.5	27	77	156	100	1019§
2	CFB -L433S	14 d post-Ecu 1200 mg	280 000	357	12.9	1.22	65	967	11	106
3	No mutations	Acute (pre-Ecu)	19 000	2373	9.2	1.9	50	411	142	478§
3	No anti-CFH Ab	21 d post-Ecu 300 mg	564 000	n.d.	12.5	0.6	76	116	46	105
4	No mutations	Acute (pre-Ecu)	107 000	768	9.6	15.2	78	556	45	439§
4	No anti-CFH Ab	14 d post-Ecu 1200 mg	161 000	342	11.7	4.77	88	260	4	59
5	CFH-S1191L	Remission (pre-Ecu)	237 000	576	10.9	8.91	93	121	72	298§
		8 d post-Ecu 300 mg	67 000	560	13.5	0.52	103	195	3	152§
		12 d post-Ecu 600 mg	119 000	588	13.6	0.59	95	214	3	43
		11 d post-Ecu 600 mg	225 000	493	12.6	0.5	n.d.	167	6	76
6	C3-K633R	Acute (pre-Ecu)	39 000	5000	7.7	1.48	123	392	93	342§
		6 d post-Ecu 600 mg	159 000	854	8.5	4.29	126	231	1	119
		6 d post-Ecu 900 mg	232 000	473	11.5	1.5	n.d.	566	11	82
7	CFH-R1210C	Acute (pre-Ecu)	46 000	1962	7	5.7	79	421	118	474§
		14 d post-Ecu 1200 mg	277 000	336	11.9	1.4	89	505	10	102
		21 d post-Ecu 1200 mg	307 000	264	12.2	1.2	83	231	32	75
8	CFI-R187Q	Acute (pre-Ecu)	94 000	869	7.2	4.5	99	534	n.d.	177§
		14 d post-Ecu 1200 mg	212 000	401	10.1	2	89	435	6	24
		1 mo post-Ecu 1200 mg	185 000	434	11.6	1.93	85	298	22	92

Ab, antibody; Ecu, eculizumab; n.d. not done.

Clinical data in the table are those recorded the same days when complement parameters were evaluated.

Limits of normal ranges (as defined in supplemental “Methods”).

Serum-induced C5b-9 deposits on ADP-activated endothelial cells (HMEC-1).

P < .01 vs control (serum from healthy subjects run in parallel, with 15 fields analyzed, as detailed in supplemental “Methods”).