Demographic and clinical characteristics at diagnosis
| Variable . | All PNH cases (N = 267) . |
|---|---|
| Follow-up, median (IQR), y | 5.9 (2.1-11.7) |
| Age at diagnosis, median (IQR), y | 46 (27-61) |
| Sex, n (%) | |
| Male | 116 (43.4) |
| Female | 151 (56.6) |
| Total PNH RBC clone, median (IQR) | 5 (0.6-25.1) |
| Type II–dominant RBC clone, n (%) | 26 (22)∗ |
| Type III–dominant RBC clone, n (%) | 92 (78)∗ |
| Total PNH WBC clone, median (IQR) | 43.9 (1.3-81.9) |
| PIGA, n (%) | |
| Wild-type | 89 (33.6) |
| Mutated | 86 (32.2) |
| Unavailable | 92 (34.2) |
| Post-AA PNH, n (%) | 85 (45.2) |
| Spontaneous remission, n (%) | 4 (1.5) |
| Thrombosis, n (%) | 56 (21) |
| Time to thrombosis, median (IQR), mo | 8 (0-58) |
| Variable . | All PNH cases (N = 267) . |
|---|---|
| Follow-up, median (IQR), y | 5.9 (2.1-11.7) |
| Age at diagnosis, median (IQR), y | 46 (27-61) |
| Sex, n (%) | |
| Male | 116 (43.4) |
| Female | 151 (56.6) |
| Total PNH RBC clone, median (IQR) | 5 (0.6-25.1) |
| Type II–dominant RBC clone, n (%) | 26 (22)∗ |
| Type III–dominant RBC clone, n (%) | 92 (78)∗ |
| Total PNH WBC clone, median (IQR) | 43.9 (1.3-81.9) |
| PIGA, n (%) | |
| Wild-type | 89 (33.6) |
| Mutated | 86 (32.2) |
| Unavailable | 92 (34.2) |
| Post-AA PNH, n (%) | 85 (45.2) |
| Spontaneous remission, n (%) | 4 (1.5) |
| Thrombosis, n (%) | 56 (21) |
| Time to thrombosis, median (IQR), mo | 8 (0-58) |
WBC, white blood cell.
Missing data (PIGA molecular information available in 91 patients, and PNH RBC clone type available in 100 patients)