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sickle-cell-disease
Sickle Cell Disease
Sickle cell disease (SCD; HbS β-globin E6V; chr11p15.4) is a haemoglobinopathy causing HbS polymerization → RBC sickling → haemolytic anaemia, vaso-occlusion, end-organ damage; hydroxyurea ↑ HbF and reduces crises; voxelotor and crizanlizumab are newer FDA-approved therapies.
Entry Metadata
| Field | Value |
|---|---|
| ID | sickle-cell-disease |
| Name | Sickle Cell Disease |
| Status | draft |
| Last reviewed | 2026-06-08 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Steinberg MH. Management of sickle cell disease. N Engl J Med. 1999;340(13):1021-1030. · PubMed 10099145
- Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342(25):1855-1865. · PubMed 10861320
- Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312(10):1033-1048. · PubMed 25205765