Table 2.
Clinical and laboratory characteristics events at of acute TMA presentation in patients with and without ADAMTS13 deficiency
| ADAMTS13 < 10% (n = 36) |
ADAMTS13 ≥ 10% (n = 8) |
Difference of proportions/ median difference (95%CI), p-value |
|
|---|---|---|---|
| Clinical signs and symptoms | |||
| Systemic, n (%) | 32 (89) | 7 (87) | 2 (− 25–28), 1 |
| Hemorrhagic, n (%) | 15 (42) | 1 (13) | 29 (− 6–65), 0.25 |
| Neurological, n (%) | 14 (39) | 4 (50) | − 11 (− 57–35), 0.97 |
| Renal, n (%) | 4 (11) | 3 (38) | − 27 (− 70–16), 0.19 |
| Cardiovascular, n (%) | 4 (11) | 0 (0) | 11 (− 7–29), 0.76 |
| Laboratory assays | |||
|
Platelet count, 109/l, median (IQR) |
14 (10–25) | 43 (34–51) | − 27 (− 37–3), 0.02 |
|
Platelet count < 30 × 109/l, n (%) |
31 (86) | 2 (25) | 61 (21–100), 0.01 |
|
Hemoglobin, g/dl, median (IQR) |
8.5 (7.2–11.2) | 7.6 (6.9–9.1) | 0.9 (− 1.0–2.9), 0.35 |
|
LDH, IU/l, median (IQR) a |
1039 (832–1761) | 1197 (836–1956) | − 113 (− 927–450), 0.67 |
|
Indirect bilirubin, mg/dl, median (IQR) b |
1.8 (1.2–2.9) | 1.8(1.2–2.3) | 0.2 (− 1.1–1.9), 0.79 |
|
Creatinine, mg/dl, median (IQR) c |
1.0 (0.8–1.3) | 1.4 (0.8–2.0) | − 0.3 (− 0.9–0.3), 0.38 |
|
Creatinine < 2.26 mg/dl, n (%) |
30 (83) | 7 (88) | − 5 (− 34–26), 1.00 |
|
eGFR, ml/min, median (IQR) d |
79 (53–107) | 50 (30–107) | 17 (− 28–55), 0.38 |
Systemic symptoms/signs: fatigue, fever, headache, jaundice (defined as total bilirubin levels ≥ 2.5 mg/dl); neurological symptoms/signs: ischemic stroke, transient ischemic attack, seizures, cognitive status alterations, personality disorders, focal neurological signs; hemorrhagic symptoms/signs: skin bleeding (purpura, ecchymosis), mucosal bleeds (including epistaxis, hematuria, meno-metrorrhagia, gastrointestinal bleeding); cardiovascular symptoms/signs: acute coronary syndrome or myocardial infarction; renal symptoms/signs: acute renal failure
aAvailable for 35 events with ADAMTS13 < 10% and 8 events with ADAMTS13 ≥ 10%
bAvailable for 26 events with ADAMTS13 < 10% and 4 events with ADAMTS13 ≥ 10%
cAvailable for 33 events with ADAMTS13 < 10% and 8 events with ADAMTS13 ≥ 10%
dAvailable for 28 events with ADAMTS13 < 10% and 8 events with ADAMTS13 ≥ 10%