Summary of findings for ADAMTS13 activity, ADAMTS13 neutralizing inhibitor, and anti-ADAMTS13 IgG and IgM antibodies
. | . | . | Presence of anti-ADAMTS13† . | . | . | ||
|---|---|---|---|---|---|---|---|
| Diagnosis . | Patients, n . | ADAMTS13 activity, range, % . | Inhibitor* . | IgG, n (%) . | IgM, n (%) . | ||
| Patients with acute acquired TMA | |||||||
| Untreated TMA, ADAMTS13 < 10% | 36 | < 3-10 | 30/36 (83%) | 35 (97) | 4 (11) | ||
| Untreated TMA, ADAMTS13 > 10% | 15 | 11-100 | 2/5 | 3 (20) | 3 (20) | ||
| Patients with thrombocytopenia, of various causes other than acute TMA or immunologic disorders | |||||||
| Thrombocytopenia | 50 | 20-100 | 0/0 | 4 (8) | 4 (8) | ||
| Systemic lupus erythematosus | 40 | 22-172 | 0/5 | 5 (13) | 7 (18) | ||
| Antiphospholipid antibody syndrome with thromboembolic complications | 55 | 32-114 | 0/10 | 3 (5) | 10 (18) | ||
| Hemophilia A with anti—factor FVIII alloantibodies | 15 | 10-91 | 0/0 | 0 | 5 (33) | ||
. | . | . | Presence of anti-ADAMTS13† . | . | . | ||
|---|---|---|---|---|---|---|---|
| Diagnosis . | Patients, n . | ADAMTS13 activity, range, % . | Inhibitor* . | IgG, n (%) . | IgM, n (%) . | ||
| Patients with acute acquired TMA | |||||||
| Untreated TMA, ADAMTS13 < 10% | 36 | < 3-10 | 30/36 (83%) | 35 (97) | 4 (11) | ||
| Untreated TMA, ADAMTS13 > 10% | 15 | 11-100 | 2/5 | 3 (20) | 3 (20) | ||
| Patients with thrombocytopenia, of various causes other than acute TMA or immunologic disorders | |||||||
| Thrombocytopenia | 50 | 20-100 | 0/0 | 4 (8) | 4 (8) | ||
| Systemic lupus erythematosus | 40 | 22-172 | 0/5 | 5 (13) | 7 (18) | ||
| Antiphospholipid antibody syndrome with thromboembolic complications | 55 | 32-114 | 0/10 | 3 (5) | 10 (18) | ||
| Hemophilia A with anti—factor FVIII alloantibodies | 15 | 10-91 | 0/0 | 0 | 5 (33) | ||