Independent prognosis factors in the 3 subcategories of PNH
| Factors . | Classic PNH, n = 109 . | AA-PNH syndrome, n = 221 . | Intermediate PNH, n = 90 . |
|---|---|---|---|
| Period, 1985 or after | 3.6 (1.3-10.3), .010 | 0.89 (0.74)† | 4.1 (1.0-16.9), .045 |
| Age at diagnosis | |||
| Between 40 and 55 y | 5.4 (1.9-15.7), <.001 | ||
| More than 40 y | 1.7 (0.11)† | ||
| More than 55 y | 21.4 (6.6-68.7), <.001 | 5.7 (1.4-23.3), .020 | |
| Thrombocytopenia | 0.16 (0.04-0.60), .007 | ||
| Androgens/danazol | 0.17 (0.03-0.88), .013 | ||
| Immunosuppressive treatment* | 0.33 (0.11-0.99), .026 | ||
| Transfusions before 1996 | 2.7 (1.3-5.6), .007 | ||
| Evolution to* | |||
| Bicytopenia or pancytopenia | 7.3 (2.5-21.5), <.001 | NA‡ | 2.5 (0.29)† |
| Thrombosis | 7.8 (3.4-17.8), <.001 | 33.0 (14.3-76.2), <.001 | 17.6 (4.5-68.5), <.001 |
| Malignant disease (MDS/AML) | 2.2 (0.68)†§ | 48.8 (15.9-149.6), <.001 | 38.5 (4.5-327.9), .003 |
| Factors . | Classic PNH, n = 109 . | AA-PNH syndrome, n = 221 . | Intermediate PNH, n = 90 . |
|---|---|---|---|
| Period, 1985 or after | 3.6 (1.3-10.3), .010 | 0.89 (0.74)† | 4.1 (1.0-16.9), .045 |
| Age at diagnosis | |||
| Between 40 and 55 y | 5.4 (1.9-15.7), <.001 | ||
| More than 40 y | 1.7 (0.11)† | ||
| More than 55 y | 21.4 (6.6-68.7), <.001 | 5.7 (1.4-23.3), .020 | |
| Thrombocytopenia | 0.16 (0.04-0.60), .007 | ||
| Androgens/danazol | 0.17 (0.03-0.88), .013 | ||
| Immunosuppressive treatment* | 0.33 (0.11-0.99), .026 | ||
| Transfusions before 1996 | 2.7 (1.3-5.6), .007 | ||
| Evolution to* | |||
| Bicytopenia or pancytopenia | 7.3 (2.5-21.5), <.001 | NA‡ | 2.5 (0.29)† |
| Thrombosis | 7.8 (3.4-17.8), <.001 | 33.0 (14.3-76.2), <.001 | 17.6 (4.5-68.5), <.001 |
| Malignant disease (MDS/AML) | 2.2 (0.68)†§ | 48.8 (15.9-149.6), <.001 | 38.5 (4.5-327.9), .003 |
Results are presented as hazard ratio estimate (95% confidence interval), P value. Results in bold are statistically significant.
Time-dependent covariate.
For nonsignificant covariate, only hazard ratio estimate and P value are shown.
Not applicable.
Only 5 cases with 1 death, 1 bone marrow transplantation, and 3 patients alive with short follow-up.