Clinical features and concomitant treatment of patients on diagnosis according to the treatment regimen
| Characteristic . | Triplet regimen (N = 90) . | Historical cohort (N = 180) . | P . |
|---|---|---|---|
| Age (y) | 45 (34-57) | 43 (30-57) | 1.00 |
| Female sex | 63 (70%) | 127 (70%) | .30 |
| Weight (kg) | 71 (60-91) | 71 (60-86) | .83 |
| Body mass index | 27.2 (22.7-32.2) | 26.6 (23-31.7) | .68 |
| Ethnicity | .39 | ||
| White | 74 | 149 | |
| African-West Indies | 10 | 25 | |
| Asian | 6 | 6 | |
| Ongoing antiplatelet agent/anticoagulation | 9 (10%) | 16 (8.9%) | .77 |
| Antiplatelet agent | 7 | 11 | |
| Anticoagulant | 2 | 5 | |
| Relapse | 12 (13.3%) | 21 (11.7%) | .70 |
| Cerebral involvement | 55 (61%) | 111 (62%) | .91 |
| Headache | 19 | 58 | |
| Confusion | 22 | 36 | |
| Seizure | 10 | 15 | |
| Coma | 2 | 5 | |
| Focal deficiency | 20 | 26 | |
| Cardiac involvement | 51 (56%) | 86 (47%) | .15 |
| Hemoglobin (g/dL) | 8.9 (7.5-10.2) | 8.6 (7.3-10.1) | .54 |
| Platelet count (×103/mm3) | 12 (10-20) | 12 (8-23) | .88 |
| LDH level xN (U/L) | 5.1 (4.0-6.5) | 3.7 (2.4-5.6) | .01 |
| Serum creatinine level (µmol/L) | 92 (71-120) | 86 (68-133) | .17 |
| GFR (mL/min per 1.73 m2) (MDRD) | 74 (51-108) | 80 (46-120) | .85 |
| ADAMTS13 activity (%) | <10% | <10% | — |
| Anti-ADAMTS13 antibodies (U/mL) | 78 (39-91) | 80 (36-100) | .44 |
| French Severity score | .37 | ||
| 0-2 | 72 (81%)* | 145 (87%)† | |
| 3-4 | 17 (19%) | 21 (13%) | |
| Immunosuppressive therapy | |||
| Corticosteroids | 88 (98%) | 166 (92%) | .10 |
| Rituximab | 90 (100%) | 123 (68%) | <.01 |
| Time between first infusion and first TPE | 2 (1-3) | 7 (4-10) | <.01 |
| Other therapies | 0 | 25 (13.9%) | <.01 |
| Twice-daily TPE | 20 | ||
| Cyclophosphamide | 4 | ||
| Splenectomy | 2 | ||
| Vincristine | 3 | ||
| Bortezomib | 1 | ||
| >1 salvage therapy | 4 |
| Characteristic . | Triplet regimen (N = 90) . | Historical cohort (N = 180) . | P . |
|---|---|---|---|
| Age (y) | 45 (34-57) | 43 (30-57) | 1.00 |
| Female sex | 63 (70%) | 127 (70%) | .30 |
| Weight (kg) | 71 (60-91) | 71 (60-86) | .83 |
| Body mass index | 27.2 (22.7-32.2) | 26.6 (23-31.7) | .68 |
| Ethnicity | .39 | ||
| White | 74 | 149 | |
| African-West Indies | 10 | 25 | |
| Asian | 6 | 6 | |
| Ongoing antiplatelet agent/anticoagulation | 9 (10%) | 16 (8.9%) | .77 |
| Antiplatelet agent | 7 | 11 | |
| Anticoagulant | 2 | 5 | |
| Relapse | 12 (13.3%) | 21 (11.7%) | .70 |
| Cerebral involvement | 55 (61%) | 111 (62%) | .91 |
| Headache | 19 | 58 | |
| Confusion | 22 | 36 | |
| Seizure | 10 | 15 | |
| Coma | 2 | 5 | |
| Focal deficiency | 20 | 26 | |
| Cardiac involvement | 51 (56%) | 86 (47%) | .15 |
| Hemoglobin (g/dL) | 8.9 (7.5-10.2) | 8.6 (7.3-10.1) | .54 |
| Platelet count (×103/mm3) | 12 (10-20) | 12 (8-23) | .88 |
| LDH level xN (U/L) | 5.1 (4.0-6.5) | 3.7 (2.4-5.6) | .01 |
| Serum creatinine level (µmol/L) | 92 (71-120) | 86 (68-133) | .17 |
| GFR (mL/min per 1.73 m2) (MDRD) | 74 (51-108) | 80 (46-120) | .85 |
| ADAMTS13 activity (%) | <10% | <10% | — |
| Anti-ADAMTS13 antibodies (U/mL) | 78 (39-91) | 80 (36-100) | .44 |
| French Severity score | .37 | ||
| 0-2 | 72 (81%)* | 145 (87%)† | |
| 3-4 | 17 (19%) | 21 (13%) | |
| Immunosuppressive therapy | |||
| Corticosteroids | 88 (98%) | 166 (92%) | .10 |
| Rituximab | 90 (100%) | 123 (68%) | <.01 |
| Time between first infusion and first TPE | 2 (1-3) | 7 (4-10) | <.01 |
| Other therapies | 0 | 25 (13.9%) | <.01 |
| Twice-daily TPE | 20 | ||
| Cyclophosphamide | 4 | ||
| Splenectomy | 2 | ||
| Vincristine | 3 | ||
| Bortezomib | 1 | ||
| >1 salvage therapy | 4 |
Data are given as median (25th-75th percentile) for quantitative variables and as n (%) for qualitative variables. Severe ADAMTS13 activity was defined as an activity <10% (normal range for ADAMTS13 activity: 50%-100%). The positivity threshold for anti-ADAMTS13 immunoglobulin G (IgG) was 15 U/mL, according to the manufacturer’s instructions (Technoclone). Cardiac involvement was defined as an increase of troponin and/or electrocardiographic abnormalities. Patients at high risk of early death of iTTP were defined by a French severity score ≥ 3 (cerebral involvement: yes = 1/no = 0, LDH: >10×ULN = 1/≤10×ULN = 0, age: >60 y = 2/>40 and ≤60 y = 1/≤40 y = 0).12
Data from 89 patients.
Data from 166 patients.