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Updated: May 1, 2026

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Sickle cell disease in India.

Roshan Colah1, Malay Mukherjee, Kanjaksha Ghosh

  • 1National Institute of Immunohaematology, Parel, Mumbai, India.

Current Opinion in Hematology
|April 10, 2014
PubMed
Summary

Sickle cell disease (SCD) in India presents varied severity, even with high fetal hemoglobin. Hydroxyurea therapy and comprehensive care show significant benefits, with newborn screening aiding disease understanding.

Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Sickle cell disease (SCD) represents a significant health challenge in India.
  • Understanding the diverse clinical presentations and genetic factors is crucial.

Purpose of the Study:

  • To review recent initiatives in India concerning SCD.
  • Focus on variable phenotypes, hydroxyurea efficacy in Asian haplotypes, and newborn screening feasibility.

Main Methods:

  • Systematic long-term follow-up of SCD patients in central India.
  • Analysis of birth cohorts to track early clinical manifestations.
  • Assessment of hydroxyurea's impact on patients with high fetal hemoglobin (HbF).

Main Results:

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  • SCD in India is not uniformly mild; early and severe anemia, septicemia are common in central India.
  • Low-dose hydroxyurea demonstrated clinical and hematological improvement in severe cases with high HbF.
  • Regional variations observed, with tribal communities in south Gujarat showing fewer severe complications.
  • Increased awareness drives demand for prenatal diagnosis.

    • Conclusions:

    • SCD severity in India is variable and not solely predicted by HbF levels.
    • Comprehensive care and hydroxyurea therapy are beneficial for SCD management.
    • Newborn screening is feasible and provides insights into the disease's natural history.
    • Government support is increasing for SCD diagnosis and care centers.