Thrombotic thrombocytopenic purpura
Description
- Thrombotic microangiopathy from severe ADAMTS13 deficiency (<10%)
- Platelet-VWF microthrombi in high-shear arterioles/capillaries -> MAHA + consumptive thrombocytopenia + ischaemic organ injury
- Haematological emergency - untreated mortality >90%
Two forms
| Immune (iTTP) | ~95% | Anti-ADAMTS13 IgG (inhibitory or clearing). Adults |
| Congenital (cTTP, Upshaw-Schulman) | ~5% | Biallelic ADAMTS13 mutations. Neonatal jaundice, childhood, or unmasked by first pregnancy |
- Target organs are those with the highest shear: brain, heart, kidney, gut, pancreas
- Renal failure is usually mild - severe renal failure points to HUS/aHUS instead
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