Baseline demographic and clinical characteristics of 87 hTTP patients, including 43 with and 44 without acute episodes during follow-up
| Characteristic . | All patients . | No TTP episodes during follow-up . | Acute TTP episodes during follow-up . | P* . | |||
|---|---|---|---|---|---|---|---|
| N . | n (%) or median [min, lq, uq, max] . | N . | n (%) or median [min, lq, uq, max] . | N . | n (%) or median [min, lq, uq, max] . | ||
| Sex | 87 | 44 | 43 | .13 | |||
| Male | 46 (53%) | 27 (61%) | 19 (44%) | ||||
| Female | 41 (47%) | 17 (39%) | 24 (56%) | ||||
| Consanguinity of parents | 67 | 9 (13%) | 32 | 6 (19%) | 35 | 3 (8.6%) | .29 |
| Family member is confirmed patient | 87 | 13 (15%) | 44 | 5 (11%) | 43 | 8 (19%) | .38 |
| Age, y, at | |||||||
| Overt disease onset | 77 | 4.6 [0.0, 0.0, 19, 70] | 35 | 4.7 [0.0, 0.0, 20, 70] | 42 | 3.6 [0.0, 0.0, 19, 68] | .80 |
| Clinical diagnosis | 85 | 18 [0.0, 4.2, 29, 70] | 42 | 20 [0.0, 9.4, 33, 70] | 43 | 15 [0.0, 3.5, 28, 68] | .12 |
| Enrollment | 87 | 26 [1.2, 16, 40, 75] | 44 | 27 [1.7, 18, 44, 75] | 43 | 26 [1.2, 14, 37, 69] | .33 |
| The last follow-up | 87 | 32 [2.1, 21, 46, 79] | 44 | 31 [5.2, 22, 48, 79] | 43 | 32 [2.1, 21, 42, 73] | .51 |
| Treatment | |||||||
| Prophylactic plasma infusions | 83 | 47 (57%) | 40 | 24 (60%) | 43 | 23 (53%) | .66 |
| Antiaggregation/anticoagulation | 79 | 21 (27%) | 37 | 12 (32%) | 42 | 9 (21%) | .31 |
| Antihypertensive medication | 79 | 25 (32%) | 37 | 14 (38%) | 42 | 11 (26%) | .33 |
| Comorbidities at enrollment | |||||||
| Arterial thrombotic disease | 83 | 30 (36%) | 41 | 19 (46%) | 42 | 11 (26%) | .07 |
| Myocardial infarction | 83 | 4 (4.8%) | 41 | 2 (4.9%) | 42 | 2 (4.8%) | 1.00 |
| Transient ischemic attack | 83 | 13 (16%) | 41 | 7 (17%) | 42 | 6 (14%) | .77 |
| Stroke | 83 | 22 (27%) | 41 | 15 (37%) | 42 | 7 (17%) | .049 |
| Other | 83 | 4 (4.8%) | 41 | 3 (7.3%) | 42 | 1 (2.4%) | .36 |
| Neurologic disorders | 83 | 18 (22%) | 41 | 13 (32%) | 42 | 5 (12%) | .035 |
| Epileptic seizure | 83 | 6 (7.2%) | 41 | 6 (15%) | 42 | 0 (0%) | .012 |
| Headache | 83 | 3 (3.6%) | 41 | 2 (4.9%) | 42 | 1 (2.4%) | .62 |
| Other neurologic disease | 83 | 12 (14%) | 41 | 8 (20%) | 42 | 4 (10%) | .23 |
| Venous thrombotic disease | 83 | 3 (3.6%) | 41 | 3 (7.3%) | 42 | 0 (0%) | .12 |
| Renal insufficiency | 83 | 26 (31%) | 41 | 11 (27%) | 42 | 15 (36%) | .48 |
| Pathologic renal parameters | 83 | 17 (20%) | 41 | 7 (17%) | 42 | 10 (24%) | .59 |
| On dialysis | 83 | 8 (10%) | 41 | 2 (4.9%) | 42 | 6 (14%) | .26 |
| Kidney transplantation | 83 | 2 (2.4%) | 41 | 0 (0%) | 42 | 2 (4.8%) | .49 |
| Neonatal hyperbilirubinemia | 83 | 36 (43%) | 41 | 16 (39%) | 42 | 20 (48%) | .51 |
| Characteristic . | All patients . | No TTP episodes during follow-up . | Acute TTP episodes during follow-up . | P* . | |||
|---|---|---|---|---|---|---|---|
| N . | n (%) or median [min, lq, uq, max] . | N . | n (%) or median [min, lq, uq, max] . | N . | n (%) or median [min, lq, uq, max] . | ||
| Sex | 87 | 44 | 43 | .13 | |||
| Male | 46 (53%) | 27 (61%) | 19 (44%) | ||||
| Female | 41 (47%) | 17 (39%) | 24 (56%) | ||||
| Consanguinity of parents | 67 | 9 (13%) | 32 | 6 (19%) | 35 | 3 (8.6%) | .29 |
| Family member is confirmed patient | 87 | 13 (15%) | 44 | 5 (11%) | 43 | 8 (19%) | .38 |
| Age, y, at | |||||||
| Overt disease onset | 77 | 4.6 [0.0, 0.0, 19, 70] | 35 | 4.7 [0.0, 0.0, 20, 70] | 42 | 3.6 [0.0, 0.0, 19, 68] | .80 |
| Clinical diagnosis | 85 | 18 [0.0, 4.2, 29, 70] | 42 | 20 [0.0, 9.4, 33, 70] | 43 | 15 [0.0, 3.5, 28, 68] | .12 |
| Enrollment | 87 | 26 [1.2, 16, 40, 75] | 44 | 27 [1.7, 18, 44, 75] | 43 | 26 [1.2, 14, 37, 69] | .33 |
| The last follow-up | 87 | 32 [2.1, 21, 46, 79] | 44 | 31 [5.2, 22, 48, 79] | 43 | 32 [2.1, 21, 42, 73] | .51 |
| Treatment | |||||||
| Prophylactic plasma infusions | 83 | 47 (57%) | 40 | 24 (60%) | 43 | 23 (53%) | .66 |
| Antiaggregation/anticoagulation | 79 | 21 (27%) | 37 | 12 (32%) | 42 | 9 (21%) | .31 |
| Antihypertensive medication | 79 | 25 (32%) | 37 | 14 (38%) | 42 | 11 (26%) | .33 |
| Comorbidities at enrollment | |||||||
| Arterial thrombotic disease | 83 | 30 (36%) | 41 | 19 (46%) | 42 | 11 (26%) | .07 |
| Myocardial infarction | 83 | 4 (4.8%) | 41 | 2 (4.9%) | 42 | 2 (4.8%) | 1.00 |
| Transient ischemic attack | 83 | 13 (16%) | 41 | 7 (17%) | 42 | 6 (14%) | .77 |
| Stroke | 83 | 22 (27%) | 41 | 15 (37%) | 42 | 7 (17%) | .049 |
| Other | 83 | 4 (4.8%) | 41 | 3 (7.3%) | 42 | 1 (2.4%) | .36 |
| Neurologic disorders | 83 | 18 (22%) | 41 | 13 (32%) | 42 | 5 (12%) | .035 |
| Epileptic seizure | 83 | 6 (7.2%) | 41 | 6 (15%) | 42 | 0 (0%) | .012 |
| Headache | 83 | 3 (3.6%) | 41 | 2 (4.9%) | 42 | 1 (2.4%) | .62 |
| Other neurologic disease | 83 | 12 (14%) | 41 | 8 (20%) | 42 | 4 (10%) | .23 |
| Venous thrombotic disease | 83 | 3 (3.6%) | 41 | 3 (7.3%) | 42 | 0 (0%) | .12 |
| Renal insufficiency | 83 | 26 (31%) | 41 | 11 (27%) | 42 | 15 (36%) | .48 |
| Pathologic renal parameters | 83 | 17 (20%) | 41 | 7 (17%) | 42 | 10 (24%) | .59 |
| On dialysis | 83 | 8 (10%) | 41 | 2 (4.9%) | 42 | 6 (14%) | .26 |
| Kidney transplantation | 83 | 2 (2.4%) | 41 | 0 (0%) | 42 | 2 (4.8%) | .49 |
| Neonatal hyperbilirubinemia | 83 | 36 (43%) | 41 | 16 (39%) | 42 | 20 (48%) | .51 |
lq, lower quartile; max, maximum; min, minimum; n, number of patients in each category; N, number of patients with information available; uq, upper quartile.
Comparison of groups with and without acute episodes during follow-up.