Table 3. Treatment and Outcome in 214 Patients with Thrombotic Microangiopathy According to ADAMTS13 Activity.
| Deficiency group (n = 160) | Detectable group (n = 54) | P Value | |
| Plasma volume, mL/kg1 | 762 (678) | 686 (759) | .15 |
| Steroids2 | 130 (81%) | 32 (59%) | .001 |
| Rituximab3 | 27 (17%) | 0 (0%) | - |
| Vincristine | 35 (22%) | 5 (9%) | .06 |
| Splenectomy | 7 (4%) | 1 (2%) | .66 |
| Time to platelet count recovery, days, median (25th–75th percentile) | 16 (7–27) | 11 (5–38) | .36 |
| Survival | 142 (89%) | 47 (87%) | .73 |
| Flare-up episode(s) | 76 (51%) | 13 (26%) | <.01 |
| Relapse4 | 28 (20%) | 7 (14%) | .37 |
| ESRD | 0 (0%) | 10 (21%) | <.0001 |
Data are presented as mean (standard deviation) or number (percent).
1
Plasma volume refers to plasma volume infused until durable complete remission.
2
Posology was 1 to 1.5 mg/kg/day for 3 weeks, with a subsequent progressive decrease within the following weeks.
3
Four 375 mg/m2 infusions were performed within 2 to 3 weeks immediately after a PE session.
4
The incidence rate (%) was calculated by dividing the number of patients who relapsed by the number of survivors.
Abbreviation: ESRD, end-stage renal disease.