Complement biomarker studies at presentation for all subjects and in the context of mutation status and response to PEX
| . | Median complement biomarkers (ng/mL) (normal range) . | ||||
|---|---|---|---|---|---|
| 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) . | |
| All patients (n = 19) | 7386 (603-30 614) | 2914 (1394-15 210) | 1098 (422-4840) | 115 (55-280) | 1237 (79-13 726) |
| Mutation present* (n = 6) | 9299 (1050-30 614) | 2571 (1640-3831) | 1480 (422-4840) | 125 (56-269) | 1353 (79-3490) |
| No mutation (n = 6) | 1756 (910-15 096) | 3338 (1394-4981) | 954 (621-1317) | 97 (55-280) | 759 (566-1391) |
| P value | .322 | .401 | .206 | .795 | .189 |
| PEX response (n = 6) | 14 945 (7386-20 443) | 3495 (1640-4680) | 1394 (1024-1920) | 141 (105-214) | 1266 (590-13 726) |
| PEX nonresponder (n = 10) | 3103 (603-30 614) | 3066 (1394-4981) | 1063 (422-4840) | 108 (55-269) | 1158 (79-3834) |
| P value | .185 | .818 | .593 | .675 | .389 |
| . | Median complement biomarkers (ng/mL) (normal range) . | ||||
|---|---|---|---|---|---|
| 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) . | |
| All patients (n = 19) | 7386 (603-30 614) | 2914 (1394-15 210) | 1098 (422-4840) | 115 (55-280) | 1237 (79-13 726) |
| Mutation present* (n = 6) | 9299 (1050-30 614) | 2571 (1640-3831) | 1480 (422-4840) | 125 (56-269) | 1353 (79-3490) |
| No mutation (n = 6) | 1756 (910-15 096) | 3338 (1394-4981) | 954 (621-1317) | 97 (55-280) | 759 (566-1391) |
| P value | .322 | .401 | .206 | .795 | .189 |
| PEX response (n = 6) | 14 945 (7386-20 443) | 3495 (1640-4680) | 1394 (1024-1920) | 141 (105-214) | 1266 (590-13 726) |
| PEX nonresponder (n = 10) | 3103 (603-30 614) | 3066 (1394-4981) | 1063 (422-4840) | 108 (55-269) | 1158 (79-3834) |
| P value | .185 | .818 | .593 | .675 | .389 |
Twelve of the 19 subjects had mutation studies performed.
Mutations studied included the following: factor H, factor I, factor B, C3, membrane cofactor protein (MCP), thrombomodulin, and CFHR3-CFHR1.