Clinical categories of patients with an initial clinical diagnosis of TTP, 1989-2008
| Clinical category . | All patients, 1989-2008 (N = 376) . | Patients with ADAMTS13 activity measurements at the time of their initial diagnosis with TTP, 1995-2008 . | |||
|---|---|---|---|---|---|
| Total (n = 261) . | ADAMTS13 activity . | ||||
| 10% or more (n = 201) . | < 10% (n = 60) . | < 5%* (n = 49) . | |||
| No. of patients (no. of patients who survived) . | |||||
| Stem cell transplantation | 23 (6) | 10 (1) | 9 (1) | 1 (0) | 0 |
| Pregnant/postpartum | 27 (25) | 15 (14) | 12 (11) | 3 (3) | 2 (2) |
| Drug association | 49 (41) | 35 (32) | 35 (32) | 0 | 0 |
| Bloody diarrhea | 28 (21) | 22 (17) | 20 (15) | 2 (2) | 2 (2) |
| Additional or alternative disorder | |||||
| Autoimmune disorder | 40 (27) | 31 (21) | 28 (18) | 3 (3) | 3 (3) |
| Systemic infection | 30 (8) | 22 (7) | 18 (5) | 4 (2) | 1 (1) |
| Systemic malignancy | 13 (2) | 10 (2) | 9 (2) | 1 (0) | 0 |
| Malignant hypertension | 6 (6) | 6 (6) | 6 (6) | 0 | 0 |
| Multiorgan failure | 11 (3) | 11 (3) | 11 (3) | 0 | 0 |
| Congenital hemolytic anemia | 1 (1) | 1 (1) | 1 (1) | 0 | 0 |
| Idiopathic | 148 (118) | 98 (79) | 52 (42) | 46 (37) | 41 (33) |
| Total | 376 (258) | 261 (178) | 201 (136) | 60 (47) | 49* (41) |
| Clinical category . | All patients, 1989-2008 (N = 376) . | Patients with ADAMTS13 activity measurements at the time of their initial diagnosis with TTP, 1995-2008 . | |||
|---|---|---|---|---|---|
| Total (n = 261) . | ADAMTS13 activity . | ||||
| 10% or more (n = 201) . | < 10% (n = 60) . | < 5%* (n = 49) . | |||
| No. of patients (no. of patients who survived) . | |||||
| Stem cell transplantation | 23 (6) | 10 (1) | 9 (1) | 1 (0) | 0 |
| Pregnant/postpartum | 27 (25) | 15 (14) | 12 (11) | 3 (3) | 2 (2) |
| Drug association | 49 (41) | 35 (32) | 35 (32) | 0 | 0 |
| Bloody diarrhea | 28 (21) | 22 (17) | 20 (15) | 2 (2) | 2 (2) |
| Additional or alternative disorder | |||||
| Autoimmune disorder | 40 (27) | 31 (21) | 28 (18) | 3 (3) | 3 (3) |
| Systemic infection | 30 (8) | 22 (7) | 18 (5) | 4 (2) | 1 (1) |
| Systemic malignancy | 13 (2) | 10 (2) | 9 (2) | 1 (0) | 0 |
| Malignant hypertension | 6 (6) | 6 (6) | 6 (6) | 0 | 0 |
| Multiorgan failure | 11 (3) | 11 (3) | 11 (3) | 0 | 0 |
| Congenital hemolytic anemia | 1 (1) | 1 (1) | 1 (1) | 0 | 0 |
| Idiopathic | 148 (118) | 98 (79) | 52 (42) | 46 (37) | 41 (33) |
| Total | 376 (258) | 261 (178) | 201 (136) | 60 (47) | 49* (41) |
The 376 patients who were enrolled in the Registry for their first episode of clinically diagnosed TTP are presented according to their clinical categories, with the number of surviving patients in parentheses. In addition, the 261 patients who had ADAMTS13 activity measured at the time of their initial diagnosis are presented separately, including the subgroups of 201 patients whose ADAMTS13 activity was ≥ 10%, the 60 patients whose ADAMTS13 activity was < 10%, and the 49 patients whose ADAMTS13 activity was < 5%.
Patients with < 5% activity (n = 49) are a subset of patients with < 10% activity (n = 60).