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pnh
Paroxysmal Nocturnal Hemoglobinuria
PNH is a clonal PIGA disorder of GPI-anchor synthesis; GPI-deficient RBCs lack CD55/CD59 → complement MAC lysis + C5a-driven thrombosis; eculizumab and ravulizumab (anti-C5 mAbs) normalize hemolysis and reduce thrombosis 90%; iptacopan (oral factor B inhibitor) also approved.
Entry Metadata
| Field | Value |
|---|---|
| ID | pnh |
| Name | Paroxysmal Nocturnal Hemoglobinuria |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Hillmen P, Hall C, Marsh JC, et al. Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med. 2004;350(6):552-559. · PubMed 14762182
- Brodsky RA, Young NS, Antonioli E, et al. Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria. Blood. 2008;111(4):1840-1847. · PubMed 18055865