Clinical and hematologic features of patients investigated
. | . | . | . | . | . | . | . | . | Size of PNH-cell population* . | . | . | . | . | . | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patients with PNH . | Sex/age, y . | Time since diagnosis, y . | Hb, g/L . | WBC, x10–9/L . | ANC, x10–9/L . | Lymph, x10–9/L . | Platelets, x10–9/L . | Retics, % . | RBCs, % . | PMN, % . | Hemoglobinuria . | Thrombosis . | Blood transfusion . | Other treatment . | |
1 | M/53 | 1 | 102 | 4.4 | 3.2 | 0.9 | 249 | 6.0 | 21 | 81 | Yes | No | Occasional | Supportive | |
2 | M/45 | 2 | 94 | 2.9 | 1.6 | 1.1 | 97 | 4.8 | 20 | 55 | Yes | No | Never | Supportive | |
3 | M/34 | 2 | 101 | 4.7 | 2.5 | 1.7 | 178 | 10.2 | 50 | 80 | Yes | No | Occasional | Supportive† | |
4 | F/54 | 10 | 81 | 3.3 | 2.4 | 0.7 | 57 | 7.1 | 36 | 98 | Yes | Hepato-portal | Never | Supportive | |
5 | F/54 | 10 | 77 | 3.8 | 1.8 | 1.5 | 193 | 6.4 | 30 | 93 | Yes | No‡ | Never | Supportive | |
6 | M/33 | 5 | 123 | 2.1 | 0.9 | 0.9 | 87 | 2.2 | 22 | 69 | Yes | No | Never | Supportive | |
7 | F/25 | 8 | 67 | 2.5 | 1.1 | 1.2 | 83 | 14.5 | 23 | 82 | Yes | No | Regular | Supportive§ | |
8 | F/53 | 7 | 121 | 3.6 | 1.6 | 1.7 | 64 | 2.1 | 51 | 96 | Yes | Budd-Chiari | Occasional | Supportive | |
9 | F/25 | 1 | 109 | 4.5 | 2.4 | 1.8 | 323 | 9.8 | 26 | 80 | Yes | No‡ | Never | Supportive | |
10 | F/45 | 5 | 86 | 8.7 | 7.5 | 1.0 | 143 | 17.0 | 75 | 99 | Yes | No | Occasional | Supportive | |
11 | M/33 | 5 | 101 | 5.6 | 3.3 | 1.8 | 262 | 6.8 | 37 | 97 | Yes | No | Occasional | Supportive§ | |
12 | F/55 | 23 | 84 | 3.9 | 2.5 | 1.0 | 185 | 10.1 | 54 | 90 | Yes | No | Regular | Supportive | |
13 | F/32 | 3 | 78 | 4.0 | 2.2 | 1.3 | 193 | 2.4 | 91 | 69 | Yes | No | Occasional | Supportive§ |
. | . | . | . | . | . | . | . | . | Size of PNH-cell population* . | . | . | . | . | . | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patients with PNH . | Sex/age, y . | Time since diagnosis, y . | Hb, g/L . | WBC, x10–9/L . | ANC, x10–9/L . | Lymph, x10–9/L . | Platelets, x10–9/L . | Retics, % . | RBCs, % . | PMN, % . | Hemoglobinuria . | Thrombosis . | Blood transfusion . | Other treatment . | |
1 | M/53 | 1 | 102 | 4.4 | 3.2 | 0.9 | 249 | 6.0 | 21 | 81 | Yes | No | Occasional | Supportive | |
2 | M/45 | 2 | 94 | 2.9 | 1.6 | 1.1 | 97 | 4.8 | 20 | 55 | Yes | No | Never | Supportive | |
3 | M/34 | 2 | 101 | 4.7 | 2.5 | 1.7 | 178 | 10.2 | 50 | 80 | Yes | No | Occasional | Supportive† | |
4 | F/54 | 10 | 81 | 3.3 | 2.4 | 0.7 | 57 | 7.1 | 36 | 98 | Yes | Hepato-portal | Never | Supportive | |
5 | F/54 | 10 | 77 | 3.8 | 1.8 | 1.5 | 193 | 6.4 | 30 | 93 | Yes | No‡ | Never | Supportive | |
6 | M/33 | 5 | 123 | 2.1 | 0.9 | 0.9 | 87 | 2.2 | 22 | 69 | Yes | No | Never | Supportive | |
7 | F/25 | 8 | 67 | 2.5 | 1.1 | 1.2 | 83 | 14.5 | 23 | 82 | Yes | No | Regular | Supportive§ | |
8 | F/53 | 7 | 121 | 3.6 | 1.6 | 1.7 | 64 | 2.1 | 51 | 96 | Yes | Budd-Chiari | Occasional | Supportive | |
9 | F/25 | 1 | 109 | 4.5 | 2.4 | 1.8 | 323 | 9.8 | 26 | 80 | Yes | No‡ | Never | Supportive | |
10 | F/45 | 5 | 86 | 8.7 | 7.5 | 1.0 | 143 | 17.0 | 75 | 99 | Yes | No | Occasional | Supportive | |
11 | M/33 | 5 | 101 | 5.6 | 3.3 | 1.8 | 262 | 6.8 | 37 | 97 | Yes | No | Occasional | Supportive§ | |
12 | F/55 | 23 | 84 | 3.9 | 2.5 | 1.0 | 185 | 10.1 | 54 | 90 | Yes | No | Regular | Supportive | |
13 | F/32 | 3 | 78 | 4.0 | 2.2 | 1.3 | 193 | 2.4 | 91 | 69 | Yes | No | Occasional | Supportive§ |
Hb indicates hemoglobin; PMN, granulocytes; Lymph, lymphocytes; Retics, reticulocytes; RBCs, red blood cells; Yes, recurrent episodes of macroscopic hemoglobinuria throughout several years of clinical history.
PMN reflects the relative size of the PNH-cell population more accurately than the percentage of PNH RBCs, 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