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A cautionary note regarding hydroxyurea in sickle cell disease

E P Vichinsky1, B H Lubin

  • 1Department of Hematology/Oncology, Children's Hospital Oakland Research Institute, CA 94609.

Blood
|February 15, 1994
PubMed
Summary

Hydroxyurea increases fetal hemoglobin in sickle cell disease (SCD) patients, but severe complications like acute chest syndrome and hemorrhage can still occur, even with elevated HbF levels. Caution is advised for hydroxyurea therapy in SCD.

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Area of Science:

  • Hematology
  • Pharmacology

Background:

  • Hydroxyurea therapy can increase fetal hemoglobin (HbF) levels, potentially improving the clinical course of sickle cell disease (SCD).
  • Unresolved issues include variable drug clearance, unpredictable responses, and the extent of reversibility of organ damage and efficacy of elevated HbF in SCD patients.

Observation:

  • Two SCD patients were treated with hydroxyurea for 1-4 years, showing a 5-10 fold increase in HbF (5-25% and 3-31%).
  • Despite HbF levels exceeding 20%, patient A experienced acute chest syndrome, and patient B had a fatal cerebral hemorrhage while on hydroxyurea therapy.

Findings:

  • Both patients experienced major SCD-related complications despite achieving significantly elevated HbF levels.
  • Hydroxyurea treatment led to pancytopenia in one patient and was associated with a fatal hemorrhage in the other.

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  • Progressive vascular changes in SCD may not be significantly reversed by elevated HbF levels alone.

    • Implications:

    • The efficacy and toxicity of hydroxyurea in SCD are not fully understood, necessitating cautious prescription.
    • Further investigation through clinical trials is crucial before widespread adoption of hydroxyurea for managing sickle cell disease.
    • Elevated HbF levels may not fully mitigate severe SCD complications or prevent vascular damage.