Clinical and Laboratory Features of Patients Studied
| Patient ID . | Age (yr) . | Sex . | Clinical Features* . | Hb† (g/dL) . | Platelet Count/ (109/L) . | Neutrophil Count†/ (109/L) . | Lymphocyte Count†/ (109/L) . | GPI-Deficient Clone (%)‡ . | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Granulocytes . | RBCs . | |||||||||
| Type II . | Type III . | |||||||||
| PNH003 | 40 | M | Hypoplastic | 16.6 | 119 | 2.5 | 1.5 | 5.9 | — | 2.2 |
| PNH008 | 29 | F | Originally hemolytic, now nonhemolytic | 14.9 | 246 | 3.6 | 2.3 | 1.4 | — | 0.5 |
| PNH012 | 34 | F | Hypoplastic | 9.6 | 276 | 5.2 | 1.5 | 45.1 | 45.3 | 0.6 |
| PNH019 | 45 | M | Hemolytic | 10.8 | 151 | 2.0 | 1.8 | 82.0 | — | 31.4 |
| PNH020 | 26 | M | Hypoplastic | 8.7 | 250 | 2.1 | 1.2 | 95.5 | — | 26.2 |
| PNH022 | 47 | F | Hemolytic | 10.2 | 173 | 1.4 | 0.6 | 93.0 | — | 20.9 |
| PNH030 | 66 | F | Spontaneous remission | 12.8 | 207 | 3.3 | 1.8 | 0.0 | — | 0.0 |
| PNH033 | 34 | F | Hemolytic | 8.7 | 55 | 1.7 | 0.6 | 99.5 | — | 79.5 |
| PNH034 | 53 | F | Hemolytic | 11.3 | 116 | 0.5 | 1.2 | 73.8 | 2.7 | 30.1 |
| PNH042 | 54 | M | Hypoplastic | 10.1 | 229 | 3.0 | 0.9 | 94.9 | 13.0 | 36.6 |
| PNH043 | 47 | M | Hypoplastic | 9.2 | 71 | 1.8 | 1.0 | 96.8 | — | 30.7 |
| PNH047 | 37 | F | Hemolytic | 12.2 | 349 | 3.2 | 1.3 | 44.1 | — | 12.4 |
| PNH048 | 28 | F | Hypoplastic | 7.1 | 13 | 0.5 | 1.2 | 33.6 | — | 16.3 |
| PNH058 | 25 | F | Hemolytic | 9.8 | 219 | 3.7 | 1.7 | 96.0 | 16.0 | 41.3 |
| PNH061 | 49 | M | Hemolytic | 6.9 | 52 | 1.8 | 1.4 | 85.0 | 13.0 | 19.0 |
| PNH063 | 37 | F | Hypoplastic | 14.0 | 163 | 7.4 | 0.8 | 6.0 | 9.0 | |
| PNH064 | 22 | M | Hypoplastic | 9.6 | 10 | 0.4 | 2.9 | 9.7 | — | 4.4 |
| PNH072 | 36 | M | Hemolytic | 12.1 | 59 | 2.2 | 1.0 | 96.4 | — | 28.9 |
| PNH079 | 54 | F | Hypoplastic | 10.9 | 185 | 1.3 | 0.7 | 95.9 | 64.1 | 20.8 |
| Patient ID . | Age (yr) . | Sex . | Clinical Features* . | Hb† (g/dL) . | Platelet Count/ (109/L) . | Neutrophil Count†/ (109/L) . | Lymphocyte Count†/ (109/L) . | GPI-Deficient Clone (%)‡ . | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Granulocytes . | RBCs . | |||||||||
| Type II . | Type III . | |||||||||
| PNH003 | 40 | M | Hypoplastic | 16.6 | 119 | 2.5 | 1.5 | 5.9 | — | 2.2 |
| PNH008 | 29 | F | Originally hemolytic, now nonhemolytic | 14.9 | 246 | 3.6 | 2.3 | 1.4 | — | 0.5 |
| PNH012 | 34 | F | Hypoplastic | 9.6 | 276 | 5.2 | 1.5 | 45.1 | 45.3 | 0.6 |
| PNH019 | 45 | M | Hemolytic | 10.8 | 151 | 2.0 | 1.8 | 82.0 | — | 31.4 |
| PNH020 | 26 | M | Hypoplastic | 8.7 | 250 | 2.1 | 1.2 | 95.5 | — | 26.2 |
| PNH022 | 47 | F | Hemolytic | 10.2 | 173 | 1.4 | 0.6 | 93.0 | — | 20.9 |
| PNH030 | 66 | F | Spontaneous remission | 12.8 | 207 | 3.3 | 1.8 | 0.0 | — | 0.0 |
| PNH033 | 34 | F | Hemolytic | 8.7 | 55 | 1.7 | 0.6 | 99.5 | — | 79.5 |
| PNH034 | 53 | F | Hemolytic | 11.3 | 116 | 0.5 | 1.2 | 73.8 | 2.7 | 30.1 |
| PNH042 | 54 | M | Hypoplastic | 10.1 | 229 | 3.0 | 0.9 | 94.9 | 13.0 | 36.6 |
| PNH043 | 47 | M | Hypoplastic | 9.2 | 71 | 1.8 | 1.0 | 96.8 | — | 30.7 |
| PNH047 | 37 | F | Hemolytic | 12.2 | 349 | 3.2 | 1.3 | 44.1 | — | 12.4 |
| PNH048 | 28 | F | Hypoplastic | 7.1 | 13 | 0.5 | 1.2 | 33.6 | — | 16.3 |
| PNH058 | 25 | F | Hemolytic | 9.8 | 219 | 3.7 | 1.7 | 96.0 | 16.0 | 41.3 |
| PNH061 | 49 | M | Hemolytic | 6.9 | 52 | 1.8 | 1.4 | 85.0 | 13.0 | 19.0 |
| PNH063 | 37 | F | Hypoplastic | 14.0 | 163 | 7.4 | 0.8 | 6.0 | 9.0 | |
| PNH064 | 22 | M | Hypoplastic | 9.6 | 10 | 0.4 | 2.9 | 9.7 | — | 4.4 |
| PNH072 | 36 | M | Hemolytic | 12.1 | 59 | 2.2 | 1.0 | 96.4 | — | 28.9 |
| PNH079 | 54 | F | Hypoplastic | 10.9 | 185 | 1.3 | 0.7 | 95.9 | 64.1 | 20.8 |
*Hypoplastic patients defined by an absence of macroscopic hemoglobinuria.
Reference ranges: hemoglobin, adult male 13.5 to 18.0 g/dL, adult female 11.5 to 16.4 g/dL; platelets 150 to 400 × 109/L; lymphocyte count 1.5 to 4.0 × 109/L; neutrophil count 2.0 to 8.0 × (109/L).
GPI-deficient clones as measured by flow cytometry. Type II RBCs are partially deficient and type III cells are completely deficient.