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thrombotic-thrombocytopenic-purpura
Thrombotic Thrombocytopenic Purpura
TTP is a life-threatening TMA caused by ADAMTS13 deficiency (<10%); ULVWF-platelet microthrombi → MAHA + thrombocytopenia + end-organ ischemia. Caplacizumab (anti-VWF; FDA 2019) + plasma exchange + rituximab is current first-line; untreated mortality ~90%.
Entry Metadata
| Field | Value |
|---|---|
| ID | thrombotic-thrombocytopenic-purpura |
| Name | Thrombotic Thrombocytopenic Purpura |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380(4):335-346. · PubMed 30625070
- George JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med. 2006;354(18):1927-1935. · PubMed 16672704
- Coppo P, Busson M, Veyradier A, et al. HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related thrombotic thrombocytopenic purpura in Caucasians. J Thromb Haemost. 2010;8(11):2466-2469. · PubMed 20735727