MVP Journal of Medical Sciences

Hemoglobin Patterns in Sickle Cell Hemoglobinopathies- A Large Prospective Study in North Maharashtra


Affiliations

  • Department of Pathology, Shri Bhausaheb Hire Government Medical College, Dhule, Maharashtra, 424005, India
  • Department of Pathology, Shri Bhausaheb Hire Government Medical College, Dhule, MUHS University Maharashtra, 424001, India

Abstract

Introduction: Sickle cell haemoglobinopathy is an inherited hemoglobinopathy resulting from a mutation occurring in betaglobin gene, on chromosome 11. The gene is prevalent in some tribes of North Maharashtra. The main aim of the study is to determine haemoglobin patterns in cases with sickle cell hemoglobinoathies in North Maharashtra using HPLC testing system. Material and Methods: This is a prospective study done over a period of 6 years. 10081 patients having positive solubility test or negative solubility test but having clinical suspicion of Sickle cell hemoglobinopathies were studied in detail and all samples were subjected for HPLC testing. Results: Prevalence of sickle cell hemoglobinopathy in this study was 70.36%. Most common pattern of haemoglobin observed was SA (89.72%). A slight female preponderance (54%) was noted. Predominant age group was paediatric (39.96%), followed by12-20yrs (33.97%). Oldest case for HbSS was 55yrs male. Predominant category affected was ST (82.05%). Conclusion: A very high prevalence of Sickle cell hemoglobinopathy was noted in this study. This is because the study was done in areas where Pawara and Bhill community resides who have a high frequency of HbS gene. Solubility test was found to be cost effective and easy screening test (Sensitivity being70.36%). HPLC found to be Rapid and accurate test for diagnosis of hemoglobinopathy and had helped in diagnosis of some rare heterozygous disorders like SA-HBQ India, SA-Hereditary persistence of foetal haemoglobin, HBD-SA. This is one of the largest and first of its kind prospective study which will help in prevention and cost effective management in targeted population.

Keywords

Hemoglobinopathies, HPLC, Solubility Test, Sickle Cell Anemia

Subject Collection

North Maharahtra, India

Subject Discipline

Pathology-Genetic hematological disorders

Full Text:

References

Herrick J. Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med.1910; 6:517–21.

Sickle cell anemia and other hemoglobinopathies. Fact Sheet No. 308. Geneva: World Health Organization; 2006.

Firth PG. Alvin HC. Sickle cell disease and anesthesia. Anesthesiology.2004; 101:766–85.

Serjeant GR, Serjeant BE. Sickle cell disease, 3rd ed. Oxford: Oxford University Press; 2001.

Weatherall D, Akinyanju O, Fucharoen S, Olivieri N, Musgrove P. Disease control priorities in developing countries.Jamison DT, editor. Oxford University Press; 2006. p. 663– 80.

Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: Rates and risk factors. N Engl J Med. 1994; 330:1639–44.

Allison AC. Protection afforded by sickle-cell trait against subtertian malarial infection. Br Med J. 1954; 1:290–4.

Esseltine DW, Baxter MR, Bevan JC. Sickle cell states and the anaesthetist. Can J Anaesth. 1988; 35:385–403.

Balgir RS. The burden of hemoglobinopathies in India and the challenges ahead. Curr Sci 2000; 79:1536–47.

Balgir RS. The genetic burden ofhemoglobinopathies with special reference to community health in India and the challeng ahead. Indian J Hematol Blood Transfus. 2002; 20:2–7.

Kate SL. Health problems of tribal population groups from state of Maharashtra. Ind J Med Sci. 2001; 5(2):99–108.

Lele RD, Solanki BR, Bhagwat RB. Hemoglobinopathies in Aurangabad region. Journal Association Physicians India.1962; 10:263.

Verma IC, Choudhury VP, Jain PK. Prevention of thalassemia: A necessity in India. Indian J Pediatr. 1992; 59:649– 54.

Manglani M, Lokeshwar MR, Vani VG, BhatiaN, Mhaskar V. NESTROFT-an effective test for b-thalassemia trait. Indian Pediatr. 1997; 34:703–8.

Wild BJ, Bain BJ. Investigation of abnormal hemoglobins and thalassemia. Lewis SM, Bain BJ, Bates I, editors. Dacie and Lewis Practical Hematology. 9th ed. London: Churchill Livingstone; 2001. p. 231–68.

Balgir RS. Spectrum of hemoglobinopathies inthe state of Orissa, India: A ten years cohort study. J Assoc Physicians India .2005; 53:1021–6.

Ajjack EA, Awooda HA, et al. Hemoglobin patterns in patients with sickle cell hemoglobinopathies. International Journal of Haematological Disorders. 2014; 1(1):8–11.

Kar BC, Devis, Clinical profile of sickle cell disease in Orissa.Indian Journal of Pediatric. 1997; 64:73–7.

Rao VR, Gorakshkar AC. Sickle cell haemoglobin, beta thalassemia and G6PD deficiency in tribes of MaharashtraIndia. Gene Geography. 1990; 4:131.

Kate SL. Health problems of trible population groups from state of Maharashtra. Ind J Med Sci. 2001; 5(2):99–108.


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