Table 4 Treatment and short- and long-term outcomes of acute TMA events in patients with and without ADAMTS13 deficiency
From: Lombardy diagnostic and therapeutic network of thrombotic microangiopathy
ADAMTS13 < 10% (n = 36) | ADAMTS13 ≥ 10% (n = 8) | Difference of proportions/ Median difference (95% CI), p-value | |
|---|---|---|---|
Treatment | |||
PEX, median (IQR) | 15 (7–20) | 5 (4–7) | 9 (2–14), 0.003 |
Corticosteroids, n (%) | 32 (88) | 6 (75) | 13 (− 25–53), 0.64 |
Rituximab, n (%) | 14 (39) | 0 (0) | 39 (15–62), 0.08 |
Outcome | |||
Median time to remission, median (IQR) a | 19 (7–24) | Na | |
Death, n (%) | 1 (3) | 2 (25) | − 22 (− 60–16), 0.14 |
Exacerbation, n (%) | 14 (39) | 0 (0) | 39 (15–62), 0.08 |
Relapse at 6 months, n (%) | 6 (17) | 0 (0) | 17 (− 3–36), 0.50 |