The initial percentage of PNH erythrocytes correlates with the percentage of cells lysed by NHS containing the factor H inhibitor rH19-20
Patient . | % PNH II + III cells; FACS* . | % Hemolysis†; NHS + rH19-20 . |
---|---|---|
PNH 1 | 33 ± 5 | 39 ± 1 |
PNH 2 | 46 ± 5 | 55 ± 2 |
PNH 3 | 48 ± 9 | 48 ± 0 |
PNH 4 | 72 ± 11 | 85 ± 7 |
Normal | 0 | 19 ± 3 |
Patient . | % PNH II + III cells; FACS* . | % Hemolysis†; NHS + rH19-20 . |
---|---|---|
PNH 1 | 33 ± 5 | 39 ± 1 |
PNH 2 | 46 ± 5 | 55 ± 2 |
PNH 3 | 48 ± 9 | 48 ± 0 |
PNH 4 | 72 ± 11 | 85 ± 7 |
Normal | 0 | 19 ± 3 |
Correlation coefficient is 0.96. Means (±SD) are shown.
PNH and normal EHs incubated with anti-CD59 antibody, followed by specific fluorescein-conjugated secondary antibody (Figure 1C).
rH19-20 used at 17 μM in 40% NHS + MgEGTA (final concentration). A414 was measured in the supernatant after 20 minutes. Hemolysis included lysis of PNH II and III cells as well as some lysis of normal cells with low CD59 (Figure 1A,C) due to fH inhibition.