Table 2.
Complement assays | Patient 1 | Patient 2 | Patient 3 | Positive control D279G | Normal controls | ||||
---|---|---|---|---|---|---|---|---|---|
D371G | E601K | I242L | |||||||
Seruma | Mutant construct | Seruma | Mutant construct | Seruma | Mutant construct | Mutant construct | Serum | Wild-type construct | |
C3 deposition on glomerular endothelial cells | + | ND | + | ND | + | ND | ND | 3/10b | ND |
Hemolysis sheep RBCs | + | ND | – | ND | + | ND | ND | 0/2 | ND |
Factor B binding to C3b (surface plasmon resonance) | ND | + | ND | – | ND | – | + | ND | – |
Factor B degradation by factor D | Degr | Degr | Degr | Degr | Degr | Degr | Degr | Degr | Degr |
Hemolysis of rabbit RBCs | ND | + | ND | – | ND | – | + | ND | – |
Soluble C5b-9 release from glomerular endothelial cells | ND | + | ND | – | ND | – | + | ND | – |
aSerum from Patients 1 and 2 was taken during eculizumab treatment. Serum from Patient 3 was taken before the start of eculizumab treatment. b3/10 indicates positive result in 3 of 10 controls. +, complement activation detected; -, complement activation was not detected; ND, Not done; RBCs, red blood cells; Degr, degradation detected.