Characteristics of PNH+ RA patients
| Patient . | Age, y/sex . | Time from diagnosis, mo . | WBC, 109/L . | Hb, g/dL . | Platelets, 109/L . | NCC in the sternum, 109/L . | Iliac bone biopsy . | PNH-type granulocytes/ RBCs, % . | Therapy . | Response to therapy . | Presentation of HLA-DR15 (DRB1 allele) . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 40/M | 58 | 2.6 | 10.0 | 30 | 68 | Hypercellular | 1.62/2.83 | Oxymetholone | (+) | – |
| 2 | 48/M | 14 | 2 | 11.9 | 88 | 50 | NT | 0.56/NT | (−) | NE | 1502 |
| 3 | 68/F | 26 | 2.4 | 7.9 | 23 | 207 | Normocellular | 1.1/4.65 | Cyclosporine | (−) | 1502 |
| 4 | 39/F | 2 | 2.6 | 7.1 | 40 | 73 | NT | 1.24/1.19 | (−) | NE | − |
| 5 | 40/F | 12 | 2.5 | 8.1 | 45 | 197 | NT | 2.41/10.01 | Cyclosporine | (+) | 1502 |
| 6 | 77/F | 50 | 3 | 7.7 | 31 | 186 | Hypocellular | 0.09/0.47 | (−) | NE | 1501/1502 |
| 7 | 73/F | 15 | 2.9 | 8.7 | 73 | 272 | Hypocellular | 0.004/0.08 | (−) | NE | 1502 |
| 8 | 60/F | 122 | 2.9 | 7.3 | 23 | 110 | NT | 0.01/0.1 | (−) | NE | 1501 |
| 9 | 68/M | 52 | 3.2 | 9.3 | 21 | 135 | Hypocellular | 0.08/0.3 | Cyclosporine | (−) | 1502 |
| 10 | 58/F | 78 | 2.9 | 7.9 | 67 | 188 | Normocellular | 0.12/0.57 | (−) | NE | 1502 |
| 11 | 79/F | 36 | 3.4 | 7.4 | 85 | 207 | Normocellular | 0.1/0.2 | (−) | NE | 1501 |
| 12 | 69/F | 85 | 3.2 | 7.9 | 21 | 139 | Hypocellular | 0.003/0.01 | Danazol | (+) | 1502 |
| 13 | 17/F | 6 | 2.1 | 9.1 | 19 | 139 | NT | 0.26/0.09 | Cyclosporine | (+) | 1501 |
| 14 | 59/M | 23 | 2.9 | 8.5 | 6 | 150 | Hypocellular | 0.16/0.13 | Cyclosporine | (+) | 1501 |
| 15 | 54/F | 1 | 2.4 | 8.2 | 38 | 222 | Hypocellular | 0.02/0.1 | Cyclosporine | (+) | 1502 |
| 16 | 51/M | 190 | 3.6 | 11.5 | 121 | 106 | Hypocellular | 0.04/0.005 | (−) | NE | 1501/1502 |
| 17 | 60/F | 1 | 4.7 | 5.9 | 15 | 53 | Hypocellular | 0.03/0.37 | Methenolone | (+) | 1501 |
| 18 | 67/F | 30 | 2.5 | 8.9 | 40 | 477 | NT | 0.45/0.36 | (−) | NE | 1502 |
| 19 | 56/M | 41 | 3.0 | 8.6 | 6 | 399 | Normocellular | 0.01/0.006 | Cyclosporine | (+) | 1501 |
| 20 | 68/F | 3 | 3.4 | 7.0 | 58 | 240 | Hypocellular | 0.02/0.01 | Cyclosporine | (+) | 1501 |
| 21 | 58/M | 47 | 2.3 | 8.0 | 4 | 358 | NT | 0.003/0.16 | Cyclosporine | (+) | 1501 |
| Patient . | Age, y/sex . | Time from diagnosis, mo . | WBC, 109/L . | Hb, g/dL . | Platelets, 109/L . | NCC in the sternum, 109/L . | Iliac bone biopsy . | PNH-type granulocytes/ RBCs, % . | Therapy . | Response to therapy . | Presentation of HLA-DR15 (DRB1 allele) . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 40/M | 58 | 2.6 | 10.0 | 30 | 68 | Hypercellular | 1.62/2.83 | Oxymetholone | (+) | – |
| 2 | 48/M | 14 | 2 | 11.9 | 88 | 50 | NT | 0.56/NT | (−) | NE | 1502 |
| 3 | 68/F | 26 | 2.4 | 7.9 | 23 | 207 | Normocellular | 1.1/4.65 | Cyclosporine | (−) | 1502 |
| 4 | 39/F | 2 | 2.6 | 7.1 | 40 | 73 | NT | 1.24/1.19 | (−) | NE | − |
| 5 | 40/F | 12 | 2.5 | 8.1 | 45 | 197 | NT | 2.41/10.01 | Cyclosporine | (+) | 1502 |
| 6 | 77/F | 50 | 3 | 7.7 | 31 | 186 | Hypocellular | 0.09/0.47 | (−) | NE | 1501/1502 |
| 7 | 73/F | 15 | 2.9 | 8.7 | 73 | 272 | Hypocellular | 0.004/0.08 | (−) | NE | 1502 |
| 8 | 60/F | 122 | 2.9 | 7.3 | 23 | 110 | NT | 0.01/0.1 | (−) | NE | 1501 |
| 9 | 68/M | 52 | 3.2 | 9.3 | 21 | 135 | Hypocellular | 0.08/0.3 | Cyclosporine | (−) | 1502 |
| 10 | 58/F | 78 | 2.9 | 7.9 | 67 | 188 | Normocellular | 0.12/0.57 | (−) | NE | 1502 |
| 11 | 79/F | 36 | 3.4 | 7.4 | 85 | 207 | Normocellular | 0.1/0.2 | (−) | NE | 1501 |
| 12 | 69/F | 85 | 3.2 | 7.9 | 21 | 139 | Hypocellular | 0.003/0.01 | Danazol | (+) | 1502 |
| 13 | 17/F | 6 | 2.1 | 9.1 | 19 | 139 | NT | 0.26/0.09 | Cyclosporine | (+) | 1501 |
| 14 | 59/M | 23 | 2.9 | 8.5 | 6 | 150 | Hypocellular | 0.16/0.13 | Cyclosporine | (+) | 1501 |
| 15 | 54/F | 1 | 2.4 | 8.2 | 38 | 222 | Hypocellular | 0.02/0.1 | Cyclosporine | (+) | 1502 |
| 16 | 51/M | 190 | 3.6 | 11.5 | 121 | 106 | Hypocellular | 0.04/0.005 | (−) | NE | 1501/1502 |
| 17 | 60/F | 1 | 4.7 | 5.9 | 15 | 53 | Hypocellular | 0.03/0.37 | Methenolone | (+) | 1501 |
| 18 | 67/F | 30 | 2.5 | 8.9 | 40 | 477 | NT | 0.45/0.36 | (−) | NE | 1502 |
| 19 | 56/M | 41 | 3.0 | 8.6 | 6 | 399 | Normocellular | 0.01/0.006 | Cyclosporine | (+) | 1501 |
| 20 | 68/F | 3 | 3.4 | 7.0 | 58 | 240 | Hypocellular | 0.02/0.01 | Cyclosporine | (+) | 1501 |
| 21 | 58/M | 47 | 2.3 | 8.0 | 4 | 358 | NT | 0.003/0.16 | Cyclosporine | (+) | 1501 |
White blood cell, hemoglobin, and platelet counts were determined at the time of diagnosis.
NCC indicates nucleated cell count; NT, not tested; and NE, not evaluable.