Clinical and hematologic features of investigated patients
| PNH patient . | Sex/age, y . | Time since diagnosis, y . | Hemoglobin level, g/L . | WBC, × 109/L . | ANC, × 109/L . | Lymphocyte count, × 109/L . | Platelet count, × 109/L . | Reticulocytes, % . | Size of PNH cell population,* % . | Thrombosis . | Blood transfusion . | Other treatment . | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| RBC . | PMN . | ||||||||||||
| 1 | M/53 | 1 | 102 | 4.4 | 3.2 | 0.9 | 249 | 6.0 | 21 | 81 | No | Occasional | Supportive |
| 2 | M/45 | 2 | 94 | 2.9 | 1.6 | 1.1 | 97 | 4.8 | 20 | 55 | No | Never | Supportive |
| 3 | M/34 | 2 | 101 | 4.7 | 2.5 | 1.7 | 178 | 10.2 | 50 | 80 | No | Occasional | Supportive† |
| 4 | F/54 | 10 | 81 | 3.3 | 2.4 | 0.7 | 57 | 7.1 | 36 | 98 | Hepatoportal | Never | Supportive |
| 5 | F/54 | 10 | 77 | 3.8 | 1.8 | 1.5 | 193 | 6.4 | 30 | 93 | No‡ | Never | Supportive |
| 6 | M/33 | 5 | 123 | 2.1 | 0.9 | 0.9 | 87 | 2.2 | 22 | 69 | No | Never | Supportive |
| 7 | F/25 | 8 | 67 | 2.5 | 1.1 | 1.2 | 83 | 14.5 | 23 | 82 | No | Regular | Supportive§ |
| 8 | F/53 | 7 | 121 | 3.6 | 1.6 | 1.7 | 64 | 2.1 | 51 | 96 | Budd-Chiari | Occasional | Supportive |
| 9 | F/25 | 1 | 109 | 4.5 | 2.4 | 1.8 | 323 | 9.8 | 26 | 80 | No‡ | Never | Supportive |
| 10 | F/45 | 5 | 86 | 8.7 | 7.5 | 1.0 | 143 | 17.0 | 75 | 99 | No | Occasional | Supportive |
| 11 | M/33 | 5 | 101 | 5.6 | 3.3 | 1.8 | 262 | 6.8 | 37 | 97 | No | Occasional | Supportive§ |
| 12 | F/55 | 23 | 84 | 3.9 | 2.5 | 1.0 | 185 | 10.1 | 54 | 90 | No | Regular | Supportive |
| 13 | F/32 | 3 | 78 | 4.0 | 2.2 | 1.3 | 193 | 2.4 | 91 | 69 | No | Occasional | Supportive§ |
| 14 | M/41 | 2 | 82 | 4.6 | 2.7 | 1.5 | 20 | 2.5 | 24 | 93 | No | Regular | Supportive |
| 15 | F/46 | 1 | 68 | 3.7 | 1.8 | 1.6 | 172 | 4.5 | 59 | 91 | No | Never | Supportive |
| 16 | M/25 | 5 | 79 | 5.0 | 3.6 | 1.0 | 164 | 14.1 | 16 | 84 | No | Regular | Supportive |
| 17 | M/38 | 6 | 78 | 7.0 | 6.3 | 0.6 | 327 | 9.5 | 83 | 99 | No‡ | Regular | Supportive§ |
| 18 | M/36 | 1 | 82 | 5.3 | 3.8 | 1.2 | 252 | 8.5 | 83 | 97 | No | Occasional | Supportive |
| 19 | M/65 | 6 | 112 | 7.7 | 6.7 | 0.8 | 203 | 6.7 | 35 | 67 | No | Occasional | Supportive |
| 20 | F/46 | 3 | 98 | 4.3 | 3.4 | 0.5 | 240 | 19.7 | 28 | 89 | No | Regular | Supportive |
| PNH patient . | Sex/age, y . | Time since diagnosis, y . | Hemoglobin level, g/L . | WBC, × 109/L . | ANC, × 109/L . | Lymphocyte count, × 109/L . | Platelet count, × 109/L . | Reticulocytes, % . | Size of PNH cell population,* % . | Thrombosis . | Blood transfusion . | Other treatment . | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| RBC . | PMN . | ||||||||||||
| 1 | M/53 | 1 | 102 | 4.4 | 3.2 | 0.9 | 249 | 6.0 | 21 | 81 | No | Occasional | Supportive |
| 2 | M/45 | 2 | 94 | 2.9 | 1.6 | 1.1 | 97 | 4.8 | 20 | 55 | No | Never | Supportive |
| 3 | M/34 | 2 | 101 | 4.7 | 2.5 | 1.7 | 178 | 10.2 | 50 | 80 | No | Occasional | Supportive† |
| 4 | F/54 | 10 | 81 | 3.3 | 2.4 | 0.7 | 57 | 7.1 | 36 | 98 | Hepatoportal | Never | Supportive |
| 5 | F/54 | 10 | 77 | 3.8 | 1.8 | 1.5 | 193 | 6.4 | 30 | 93 | No‡ | Never | Supportive |
| 6 | M/33 | 5 | 123 | 2.1 | 0.9 | 0.9 | 87 | 2.2 | 22 | 69 | No | Never | Supportive |
| 7 | F/25 | 8 | 67 | 2.5 | 1.1 | 1.2 | 83 | 14.5 | 23 | 82 | No | Regular | Supportive§ |
| 8 | F/53 | 7 | 121 | 3.6 | 1.6 | 1.7 | 64 | 2.1 | 51 | 96 | Budd-Chiari | Occasional | Supportive |
| 9 | F/25 | 1 | 109 | 4.5 | 2.4 | 1.8 | 323 | 9.8 | 26 | 80 | No‡ | Never | Supportive |
| 10 | F/45 | 5 | 86 | 8.7 | 7.5 | 1.0 | 143 | 17.0 | 75 | 99 | No | Occasional | Supportive |
| 11 | M/33 | 5 | 101 | 5.6 | 3.3 | 1.8 | 262 | 6.8 | 37 | 97 | No | Occasional | Supportive§ |
| 12 | F/55 | 23 | 84 | 3.9 | 2.5 | 1.0 | 185 | 10.1 | 54 | 90 | No | Regular | Supportive |
| 13 | F/32 | 3 | 78 | 4.0 | 2.2 | 1.3 | 193 | 2.4 | 91 | 69 | No | Occasional | Supportive§ |
| 14 | M/41 | 2 | 82 | 4.6 | 2.7 | 1.5 | 20 | 2.5 | 24 | 93 | No | Regular | Supportive |
| 15 | F/46 | 1 | 68 | 3.7 | 1.8 | 1.6 | 172 | 4.5 | 59 | 91 | No | Never | Supportive |
| 16 | M/25 | 5 | 79 | 5.0 | 3.6 | 1.0 | 164 | 14.1 | 16 | 84 | No | Regular | Supportive |
| 17 | M/38 | 6 | 78 | 7.0 | 6.3 | 0.6 | 327 | 9.5 | 83 | 99 | No‡ | Regular | Supportive§ |
| 18 | M/36 | 1 | 82 | 5.3 | 3.8 | 1.2 | 252 | 8.5 | 83 | 97 | No | Occasional | Supportive |
| 19 | M/65 | 6 | 112 | 7.7 | 6.7 | 0.8 | 203 | 6.7 | 35 | 67 | No | Occasional | Supportive |
| 20 | F/46 | 3 | 98 | 4.3 | 3.4 | 0.5 | 240 | 19.7 | 28 | 89 | No | Regular | Supportive |
All patients experienced recurrent episodes of hemoglobinuria.
WBC indicates white blood count; ANC, absolute neutrophil count; RBC, red blood cells; PMN, granulocytes.
The percentage of PNH PMN reflects the relative size of the PNH cell population more accurately than the percentage of PNH RBC because the latter will be grossly underestimated as a consequence of selective hemolysis; this may be further complicated by blood transfusion.
Previous prednisone treatment.
Recurrent attacks of severe abdominal pain but no documented thrombosis.
Previous antithymocyte globulin treatment.