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Sickle cell disease in India.

Dipika Mohanty1, Malay B Mukherjee

  • 1Institute of Immunohematology, Indian Council of Medical Research, Parel, Mumbai, India. mohanty@bom5vsml.net.in

Current Opinion in Hematology
|February 15, 2002
PubMed
Summary

Sickle cell anemia in India presents milder symptoms, with vaso-occlusive crises peaking in the rainy season and winter. Alpha thalassemia and iron deficiency influence disease severity, while simple interventions can reduce crises.

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Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Sickle cell anemia (SCA) clinical manifestations vary globally.
  • Indian SCA patients exhibit milder phenotypes compared to African and Jamaican cohorts.
  • Vaso-occlusive crises (VOC) are a primary concern for SCA patients.

Purpose of the Study:

  • To describe the clinical characteristics of sickle cell anemia in India.
  • To identify factors influencing disease severity and incidence of VOC.
  • To outline management and prevention strategies for SCA in the Indian context.

Main Methods:

  • Observational study analyzing clinical data of sickle cell anemia patients in India.
  • Correlation of VOC incidence with seasonal variations.
  • Assessment of the role of alpha thalassemia and iron deficiency in disease amelioration.

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Main Results:

  • Sickle cell anemia in India is generally milder.
  • Vaso-occlusive crises show peak incidence during the rainy season and winter.
  • Iron deficiency is prevalent in both SCA patients and carriers (heterozygotes).
  • Alpha thalassemia acts as a significant epistatic factor, ameliorating SCA severity.

Conclusions:

  • Simple preventive measures like vaccination, hydration, and temperature regulation can decrease VOC frequency.
  • Premarital counseling and prenatal diagnosis are crucial for reducing the incidence of homozygous sickle cell anemia births.
  • Understanding genetic modifiers like alpha thalassemia is key to managing SCA in India.