Sickle Cell Disease
Hematology · ~5% of blueprint- Pathophysiology
- HbS beta-globin mutation (Glu->Val, codon 6) from both parents -> RBC sickling under stress -> vaso-occlusion & hemolysis.
- Signs & Symptoms
- Painful vaso-occlusive crises, dactylitis, acute chest syndrome, splenic sequestration, infection risk.
- Gold Standard Diagnosis
- Hemoglobin electrophoresis, HPLC or IEF confirms HbS; universal newborn screening; smear not confirmatory.
- First-line Treatment
- Prompt analgesia (parenteral opioids if severe); O2 only if SpO2 <95%; fluids at maintenance rate; hydroxyurea; vaccinate.
Drug cardsHydroxyurea
High-Yield Pearl
Hydroxyurea raises fetal hemoglobin (HbF) and reduces the frequency of crises.
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Immunology & Genetics
Physiology
Sources
Reviewed September 2026- Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014 — NHLBI / NIH, 2014
- American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain — American Society of Hematology, 2020
- StatPearls: Sickle Cell Anemia — StatPearls Publishing / NCBI Bookshelf, 2026
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