Atlas One · Host Biology · Scale 07 — System

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%.

Also known as: TTP, thrombotic thrombocytopenic purpura, iTTP, immune TTP, Upshaw-Schulman syndrome, congenital TTP, hereditary TTP, thrombotic microangiopathy TTP, MAHA TTP

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
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%.