Trends in complement levels and hemostasis data during hospitalization
| . | Normal range . | D12 . | D18 . | D25 . | D40 . |
|---|---|---|---|---|---|
| Complement | |||||
| CH50 (%) | 70-130 | 36 | 0 | 0 | <10 |
| C3 (mg/L) | 660-1250 | 700 | 497 | 817 | 659 |
| C4 (mg/L) | 93-380 | 166 | 172 | 328 | 231 |
| Factor H (%) | 65-140 | 74 | 74 | 87 | |
| Factor I (%) | 70-130 | 92 | 81 | 91 | |
| CD46 (MFI) | 13-25 | 9 | |||
| Anti-FH antibodies | Negative | Negative | Negative | ||
| Free eculizumab (µg/mL) | 428 | 415 | 81 | ||
| sC5b9 (ng/mL) | <300 | 664 | 121 | 314 | 212 |
| Hematology/hemostasis | |||||
| Reticulocytes (109/L) | 25-100 | 286 | 340 | 183 | |
| Prothombin (factor II) (%) | 70-150 | 95 | 147 | >150 | >150 |
| Prothrombin rate (%) | 70-100 | 97 | 112 | 97 | 106 |
| Fibrinogene (g/L) | 1.5-3.5 | 2.6 | 1.7 | 1.2 | 1.8 |
| D-dimer (ng/L) | <500 | 2231 | 336 | 885 | |
| . | Normal range . | D12 . | D18 . | D25 . | D40 . |
|---|---|---|---|---|---|
| Complement | |||||
| CH50 (%) | 70-130 | 36 | 0 | 0 | <10 |
| C3 (mg/L) | 660-1250 | 700 | 497 | 817 | 659 |
| C4 (mg/L) | 93-380 | 166 | 172 | 328 | 231 |
| Factor H (%) | 65-140 | 74 | 74 | 87 | |
| Factor I (%) | 70-130 | 92 | 81 | 91 | |
| CD46 (MFI) | 13-25 | 9 | |||
| Anti-FH antibodies | Negative | Negative | Negative | ||
| Free eculizumab (µg/mL) | 428 | 415 | 81 | ||
| sC5b9 (ng/mL) | <300 | 664 | 121 | 314 | 212 |
| Hematology/hemostasis | |||||
| Reticulocytes (109/L) | 25-100 | 286 | 340 | 183 | |
| Prothombin (factor II) (%) | 70-150 | 95 | 147 | >150 | >150 |
| Prothrombin rate (%) | 70-100 | 97 | 112 | 97 | 106 |
| Fibrinogene (g/L) | 1.5-3.5 | 2.6 | 1.7 | 1.2 | 1.8 |
| D-dimer (ng/L) | <500 | 2231 | 336 | 885 | |
Eculizumab was administrated at days 13, 19, and 40 after onasemnogene abeparvovec infusion.