ADAMTS13 deficiency and thrombotic thrombocytopenia purpura (TTP).
| ADAMTS13 Plasma Activity Absent* . | Clinical Presentation . |
|---|---|
| * < 5% of normal | |
| +Autoantibodies may also be present | |
| ADAMTS13 mutations | Familial TTP; chronic relapsing TTP |
| Disease presentation in infancy/childhood | |
| Disease presentation delayed | |
| Autoantibodies against ADAMTS13 | Acquired idiopathic TTP |
| Transient | Single episode TTP |
| Recurrent | Recurrent (intermittent) TTP |
| Thienopyridine-associated | Ticlopidine/clopidogrel-TTP |
| ADAMTS13 transient production or survival (?) defect | Acquired idiopathic TTP (?) |
| Pregnancy+ | Pregnancy-associated TTP |
| ADAMTS13 Plasma Activity Absent* . | Clinical Presentation . |
|---|---|
| * < 5% of normal | |
| +Autoantibodies may also be present | |
| ADAMTS13 mutations | Familial TTP; chronic relapsing TTP |
| Disease presentation in infancy/childhood | |
| Disease presentation delayed | |
| Autoantibodies against ADAMTS13 | Acquired idiopathic TTP |
| Transient | Single episode TTP |
| Recurrent | Recurrent (intermittent) TTP |
| Thienopyridine-associated | Ticlopidine/clopidogrel-TTP |
| ADAMTS13 transient production or survival (?) defect | Acquired idiopathic TTP (?) |
| Pregnancy+ | Pregnancy-associated TTP |