Specification, clinical presentation, and treatment of acute TTP episodes during follow-up
| Variable . | All episodes . | Prophylactic treatment . | P* . | ||||
|---|---|---|---|---|---|---|---|
| . | . | No . | Yes . | ||||
| N . | Median [min, lq, uq, max] or n (%) . | N . | Median [min, lq, uq, max] or n (%) . | N . | Median [min, lq, uq, max] or n (%) . | ||
| Onset of symptoms and medical care | |||||||
| Interval, d | 131 | 0.0 [0.0, 0.0, 0.0, 4.0] | 40 | 0.0 [0.0, 0.0, 0.0, 1.0] | 91 | 0.0 [0.0, 0.0, 0.0, 4.0] | .09 |
| On the same day | 131 | 104 (79%) | 40 | 35 (88%) | 91 | 69 (76%) | .16 |
| Duration of an episode, d | 128 | 4.0 [0.0, 2.0, 5.0, 43] | 39 | 4.0 [0.0, 3.0, 6.0, 36] | 89 | 3.0 [0.0, 2.0, 5.0, 43] | .06 |
| Trigger(s) of acute TTP episode† | 131 | 87 (66%) | 40 | 31 (77%) | 91 | 56 (62%) | .11 |
| Infection | 86 | 65 (76%) | 31 | 28 (90%) | 55 | 37 (67%) | .019 |
| Alcohol consumption | 86 | 5 (5.8%) | 31 | 1 (3.2%) | 55 | 4 (7.3%) | .65 |
| Pregnancy | 87 | 6 (6.9%) | 31 | 0 (0%) | 56 | 6 (11%) | .08 |
| Other | 85 | 11 (13%) | 30 | 3 (10%) | 55 | 8 (15%) | .74 |
| Clinical presentation† | |||||||
| Fever | 124 | 28 (23%) | 37 | 9 (24%) | 87 | 19 (22%) | .82 |
| Petechiae or other bleeding | 128 | 56 (44%) | 40 | 17 (43%) | 88 | 39 (44%) | 1.00 |
| Gastrointestinal symptoms | 128 | 38 (30%) | 40 | 10 (25%) | 88 | 28 (32%) | .53 |
| Jaundice | 128 | 18 (14%) | 40 | 7 (17%) | 88 | 11 (13%) | .58 |
| Dark urine | 127 | 15 (12%) | 40 | 3 (7.5%) | 87 | 12 (14%) | .39 |
| Presence of acute renal failure | 129 | 9 (7.0%) | 40 | 1 (2.5%) | 89 | 8 (9.0%) | .27 |
| Neurologic symptoms | 129 | 28 (22%) | 40 | 9 (22%) | 89 | 19 (21%) | 1.00 |
| Epileptic seizure | 28 | 0 (0%) | 9 | 0 (0%) | 19 | 0 (0%) | — |
| Transient ischemic attack | 28 | 9 (32%) | 9 | 5 (56%) | 19 | 4 (21%) | .10 |
| Stroke | 28 | 11 (39%) | 9 | 3 (33%) | 19 | 8 (42%) | 1.00 |
| Other | 28 | 19 (68%) | 9 | 8 (89%) | 19 | 11 (58%) | .20 |
| Treatment of acute episode‡ | 130 | 119 (92%) | 40 | 35 (88%) | 90 | 84 (93%) | .31 |
| Fresh frozen plasma | 119 | 73 (61%) | 35 | 28 (80%) | 84 | 45 (54%) | .008 |
| Solvent/detergent plasma | 119 | 34 (29%) | 35 | 4 (11%) | 84 | 30 (36%) | .008 |
| Cryo-poor plasma | 119 | 8 (6.7%) | 35 | 0 (0%) | 84 | 8 (10%) | .10 |
| Plasma-derived factor VIII product | 119 | 10 (8.4%) | 35 | 0 (0%) | 84 | 10 (12%) | .033 |
| Other | 119 | 6 (5.0%) | 35 | 3 (8.6%) | 84 | 3 (3.6%) | .36 |
| Variable . | All episodes . | Prophylactic treatment . | P* . | ||||
|---|---|---|---|---|---|---|---|
| . | . | No . | Yes . | ||||
| N . | Median [min, lq, uq, max] or n (%) . | N . | Median [min, lq, uq, max] or n (%) . | N . | Median [min, lq, uq, max] or n (%) . | ||
| Onset of symptoms and medical care | |||||||
| Interval, d | 131 | 0.0 [0.0, 0.0, 0.0, 4.0] | 40 | 0.0 [0.0, 0.0, 0.0, 1.0] | 91 | 0.0 [0.0, 0.0, 0.0, 4.0] | .09 |
| On the same day | 131 | 104 (79%) | 40 | 35 (88%) | 91 | 69 (76%) | .16 |
| Duration of an episode, d | 128 | 4.0 [0.0, 2.0, 5.0, 43] | 39 | 4.0 [0.0, 3.0, 6.0, 36] | 89 | 3.0 [0.0, 2.0, 5.0, 43] | .06 |
| Trigger(s) of acute TTP episode† | 131 | 87 (66%) | 40 | 31 (77%) | 91 | 56 (62%) | .11 |
| Infection | 86 | 65 (76%) | 31 | 28 (90%) | 55 | 37 (67%) | .019 |
| Alcohol consumption | 86 | 5 (5.8%) | 31 | 1 (3.2%) | 55 | 4 (7.3%) | .65 |
| Pregnancy | 87 | 6 (6.9%) | 31 | 0 (0%) | 56 | 6 (11%) | .08 |
| Other | 85 | 11 (13%) | 30 | 3 (10%) | 55 | 8 (15%) | .74 |
| Clinical presentation† | |||||||
| Fever | 124 | 28 (23%) | 37 | 9 (24%) | 87 | 19 (22%) | .82 |
| Petechiae or other bleeding | 128 | 56 (44%) | 40 | 17 (43%) | 88 | 39 (44%) | 1.00 |
| Gastrointestinal symptoms | 128 | 38 (30%) | 40 | 10 (25%) | 88 | 28 (32%) | .53 |
| Jaundice | 128 | 18 (14%) | 40 | 7 (17%) | 88 | 11 (13%) | .58 |
| Dark urine | 127 | 15 (12%) | 40 | 3 (7.5%) | 87 | 12 (14%) | .39 |
| Presence of acute renal failure | 129 | 9 (7.0%) | 40 | 1 (2.5%) | 89 | 8 (9.0%) | .27 |
| Neurologic symptoms | 129 | 28 (22%) | 40 | 9 (22%) | 89 | 19 (21%) | 1.00 |
| Epileptic seizure | 28 | 0 (0%) | 9 | 0 (0%) | 19 | 0 (0%) | — |
| Transient ischemic attack | 28 | 9 (32%) | 9 | 5 (56%) | 19 | 4 (21%) | .10 |
| Stroke | 28 | 11 (39%) | 9 | 3 (33%) | 19 | 8 (42%) | 1.00 |
| Other | 28 | 19 (68%) | 9 | 8 (89%) | 19 | 11 (58%) | .20 |
| Treatment of acute episode‡ | 130 | 119 (92%) | 40 | 35 (88%) | 90 | 84 (93%) | .31 |
| Fresh frozen plasma | 119 | 73 (61%) | 35 | 28 (80%) | 84 | 45 (54%) | .008 |
| Solvent/detergent plasma | 119 | 34 (29%) | 35 | 4 (11%) | 84 | 30 (36%) | .008 |
| Cryo-poor plasma | 119 | 8 (6.7%) | 35 | 0 (0%) | 84 | 8 (10%) | .10 |
| Plasma-derived factor VIII product | 119 | 10 (8.4%) | 35 | 0 (0%) | 84 | 10 (12%) | .033 |
| Other | 119 | 6 (5.0%) | 35 | 3 (8.6%) | 84 | 3 (3.6%) | .36 |
n, number of episodes in each category; N, number of episodes with information available.
Comparison of groups on and not on prophylactic treatment.
Listings are not additive; multiple entries are possible.
For 11 of the 131 acute episodes, patients received no treatment; for 1 episode, no treatment details were documented.