Relation of presenting features and clinical outcomes of 142 patients with clinically diagnosed TTP-HUS to clinical categories
Clinical category, (no. of patients) . | Severe neurologic abnormalities, % (proportion) . | Acute renal failure, % (proportion) . | Response, % (proportion) . | TTP-HUS-associated death, % (proportion) . | All death, % (proportion) . | Relapse, % (proportion)* . |
|---|---|---|---|---|---|---|
| Stem cell transplantation (7) | 57 (4/7) | 0 (0/7) | 0 (0/7) | 86 (6/7) | 100 (7/7) | 0 (0/1) |
| Pregnancy/postpartum (10) | 20 (2/10) | 70 (7/10) | 90 (9/10) | 10 (1/10) | 10 (1/10) | 11 (1/9) |
| Drug associated (21) | 24 (5/21) | 67 (14/21) | 86 (18/21) | 10 (2/21) | 43 (9/21) | 5 (1/19) |
| Bloody diarrhea (10) | 60 (6/10) | 70 (7/10) | 80 (8/10) | 40 (4/10) | 40 (4/10) | 0 (0/6) |
| Additional/alternative disorders (46) | 65 (30/46) | 46 (21/46) | 28 (13/46) | 61 (28/46) | 76 (35/46) | 6 (1/18) |
| Idiopathic (48)† | 52 (25/48) | 25 (12/48) | 79 (38/48) | 21 (10/48) | 31 (15/48) | 21 (8/39) |
Clinical category, (no. of patients) . | Severe neurologic abnormalities, % (proportion) . | Acute renal failure, % (proportion) . | Response, % (proportion) . | TTP-HUS-associated death, % (proportion) . | All death, % (proportion) . | Relapse, % (proportion)* . |
|---|---|---|---|---|---|---|
| Stem cell transplantation (7) | 57 (4/7) | 0 (0/7) | 0 (0/7) | 86 (6/7) | 100 (7/7) | 0 (0/1) |
| Pregnancy/postpartum (10) | 20 (2/10) | 70 (7/10) | 90 (9/10) | 10 (1/10) | 10 (1/10) | 11 (1/9) |
| Drug associated (21) | 24 (5/21) | 67 (14/21) | 86 (18/21) | 10 (2/21) | 43 (9/21) | 5 (1/19) |
| Bloody diarrhea (10) | 60 (6/10) | 70 (7/10) | 80 (8/10) | 40 (4/10) | 40 (4/10) | 0 (0/6) |
| Additional/alternative disorders (46) | 65 (30/46) | 46 (21/46) | 28 (13/46) | 61 (28/46) | 76 (35/46) | 6 (1/18) |
| Idiopathic (48)† | 52 (25/48) | 25 (12/48) | 79 (38/48) | 21 (10/48) | 31 (15/48) | 21 (8/39) |
Presenting features, clinical outcomes, and clinical categories are defined in “Patients and methods.” There was no difference in the relative frequency of severe neurologic abnormalities (P = .814). Patients with idiopathic TTP-HUS had a lower relative frequency of acute renal failure, a higher response rate, a lower TTP-HUS-associated death rate, a lower overall death rate, and a higher relative frequency of relapse compared with patients in other categories (P < .05).
The denominator is the number of patients who achieved a remission.
Patients in the idiopathic clinical category were compared with patients in all other categories (idiopathic versus the combined categories of other patients).