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Hemoglobin SS disease with crisis

Last edited: 4/15/2026

Overview

Hemoglobin SS disease, also known as sickle cell anemia, is a genetic disorder characterized by the presence of abnormal hemoglobin S, leading to chronic hemolytic anemia and episodic vaso-occlusive crises causing severe pain and potential organ damage 1.

Diagnosis

  • Clinical Presentation: Recurrent episodes of pain, pallor, jaundice, and fatigue 1.
  • Laboratory Tests: Hemoglobin electrophoresis confirming HbSS genotype 1.
  • Blood Smear: Presence of sickle-shaped red blood cells 1.
  • Genetic Testing: Confirmatory molecular analysis for HbS mutation 1.

    • Management

  • First-Line Treatments:
    • - Pain Management: Opioids (e.g., morphine) for acute pain crises 1. - Hydration: Intravenous fluids to maintain hydration 1.
  • Adjunctive Treatments:
    • - Hydroxyurea: To reduce vaso-occlusive crises and hospitalizations (typically 15-20 mg/kg/day) 1. - Blood Transfusions: For acute complications like stroke or severe anemia 1. - Antibiotics: Prophylactic penicillin to prevent infections, especially in children 1.

    Special Populations

  • Pregnancy: Close monitoring for complications such as preeclampsia, sepsis, and obstetric emergencies; consider exchange transfusions for severe anemia 1.
  • Pediatrics: Early initiation of hydroxyurea to reduce complications; regular vaccinations and prophylactic antibiotics 1.
  • Elderly: Increased vigilance for chronic organ damage and management of comorbidities; individualized pain management strategies 1.
  • Comorbidities: Tailored management focusing on organ protection and crisis prevention; multidisciplinary care approach recommended 1.

    • Key Recommendations

  • Initiate hydroxyurea therapy in stable adults to reduce vaso-occlusive crises (Evidence: Strong 1).
  • Use prophylactic antibiotics in children to prevent infections (Evidence: Strong 1).
  • Consider blood transfusions for severe complications such as acute chest syndrome or stroke (Evidence: Moderate 1).

    • References

    1 Gannon F. Mismatched expectations. EMBO reports 2021. link

    Original source

    1. [1]
      Mismatched expectations. — Gannon F EMBO reports (2021)
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