ISSN (Online): 1694-4674
Annals of Medicine and Medical Sciences Annals of Medicine and Medical Sciences
Submit Manuscript
  1. Home
  2. Volume 04 (2025), Version 4
  3. Fetal Hemoglobin (HbF) as a Genetic Modulator in Various Clinical Phen…
Original Article Open Access

Fetal Hemoglobin (HbF) as a Genetic Modulator in Various Clinical Phenotypes of Sickle Cell Anemia in Tribal and Non-Tribal Ethnic Group of Saurashtra Region in Gujarat

, , ,
Annals of Medicine and Medical Sciences Volume 04 (2025), Version 4 April 24, 2025 pp. 223 - 227
126 69
Download PDF
PDF XML
Abstract

Objectives: To compare fetal hemoglobin (HbF) levels and clinical manifestations between Tribal and non-Tribal Sickle Cell Anemia (SCA) patients in Gujarat's Saurashtra region and investigate HbF's role in modulating disease severity. Methods: This retrospective study analyzed 68 SCA patients (33 Tribal, 35 non-Tribal) at a tertiary care center from January 2023-2024. HbF levels were measured via high-performance liquid chromatography, and clinical data on Acute Chest Syndrome (ACS), Vaso-occlusive Events (VOE), Pain Crisis, and hospitalization duration were collected from medical records. Results: Tribal patients exhibited significantly higher mean HbF levels compared to non-Tribal patients (17.8% vs. 13.0%, p<0.01). This correlated with lower incidence of ACS (36% vs. 65%, p=0.0176) and VOE (39% vs. 77%, p=0.0016) among Tribal patients. Pain Crisis incidence showed no significant difference between groups (36% vs. 45%, p=0.4535). Notably, Tribal patients experienced shorter mean hospital stays (2.12 vs. 3.37 days, p<0.01). Conclusion: Higher HbF levels in Tribal SCA patients correlate with reduced ACS and VOE incidence and shorter hospitalizations, suggesting a protective effect. These findings enhance understanding of SCA heterogeneity across ethnic groups and may inform development of population-specific management approaches. Further investigation into genetic and environmental factors underlying these differences is warranted.

References
  1. Piel, F. B., Patil, A. P., Howes, R. E., Nyangiri, O. A., Gething, P. W., Williams, T. N., ... & Hay, S. I. (2013). Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nature Communications, 4(1), 1-9.
  2. Colah, R. B., Mukherjee, M. B., Martin, S., & Ghosh, K. (2015). Sickle cell disease in tribal populations in India. Indian Journal of Medical Research, 141(5), 509-515.
  3. Rees, D. C., Williams, T. N., & Gladwin, M. T. (2010). Sickle-cell disease. The Lancet, 376(9757), 2018-2031.
  4. Steinberg, M. H., Chui, D. H., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014). Fetal hemoglobin in sickle cell anemia: a glass half full? Blood, 123(4), 481-485.
  5. Akinsheye, I., Alsultan, A., Solovieff, N., Ngo, D., Baldwin, C. T., Sebastiani, P., ... & Steinberg, M. H. (2011). Fetal hemoglobin in sickle cell anemia. Blood, 118(1), 19-27.
  6. Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P. (1994). Mortality in sickle cell disease--life expectancy and risk factors for early death. New England Journal of Medicine, 330(23), 1639-1644.
  7. Alsultan, A., Alabdulaali, M. K., Griffin, P. J., AlSuliman, A. M., Ghabbour, H. A., Sebastiani, P., ... & Steinberg, M. H. (2014). Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab-Indian haplotype is not benign. British Journal of Haematology, 164(4), 597-604.
  8. Kar, B. C., Satapathy, R. K., Kulozik, A. E., Kulozik, M., Sirr, S., Serjeant, B. E., & Serjeant, G. R. (2014). Sickle cell disease in Orissa State, India. The Lancet, 2(8327), 1198-1201.
  9. Piel, F. B., Patil, A. P., Howes, R. E., Nyangiri, O. A., Gething, P. W., Williams, T. N., ... & Hay, S. I. (2013). Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nature Communications, 4(1), 1-9.
  10. Meier, E. R., Fasano, R. M., & Levett, P. R. (2017). A systematic review of the literature for severity predictors in children with sickle cell anemia. Blood Cells, Molecules, and Diseases, 65, 86-94.
  11. Chakravorty, S., & Williams, T. N. (2015). Sickle cell disease: A neglected chronic disease of increasing global health importance. Archives of Disease in Childhood, 100(1), 48-53.
Author Resources