Share This Article:

Incidence of Sickle Cell Anaemia and Thalassaemia in Central India

Abstract Full-Text HTML Download Download as PDF (Size:643KB) PP. 71-80
DOI: 10.4236/ojbd.2012.24014    5,655 Downloads   11,962 Views   Citations

ABSTRACT

Haemoglobinopathies are group of diseases characterized by abnormalities both quantitative and qualitative in the synthesis of haemoglobin. Haemoglobinopathies consist of sickle cell anaemia (SCA), thalassaemia (βT) and variant haemoglobins. In India, they are responsible for the largest number of genetic disorders and hence are of great public health hazardous. In India major concerned haemoglobinopathic disorders are sickle cell anaemia and β-thalassaemia. Of the several abnormal haemoglobin molecules, four which are widely prevalent in India include: HbS, HbβT, HbE and HbD. Examination of 6463 individuals showed high incidences for haemoglobin variants, HbS and HbβT in different ethnic groups, the frequency being varies from 0% - 20% and 0% - 9% respectively. The frequency of HbS in Brahmins is 4.17%, in Kalar 5.41%, in Rajput 2.04%, in Muslims 3.73% in Maratha 2.08% in Bania 9.09% while in Teli it is 3.65%. Among the Scheduled castes and Nomadic tribal groups HbS ranges from 1% - 12%; in backward caste categories it varies from 3% - 16%; while in Scheduled tribes it ranges from 0% - 20%. The high magnitude of sickle cell trait has been noticed in the Pardhan (20.31%) followed by the Marar (16.10%), the Dhiwar (11.90%), the Gond (11.89%), the Mahar (11.81%) and the Bania (9.90%). A considerable high frequency (9.27%) of β-thalassaemia has been observed among the Sindhi population. Sporadic occurrence of HbβT and HbD among other communities suggested the gradual spread of the genes into the region. The present findings in 11 communities with the thalassaemia syndrome suggest that the β-thalassaemia is accompanied by raised level of HbA2. Unusual greater mean RBC and WBC suggest the high concentration of hypochromic microcytosis in anaemia. The mean MCV and MCH in HbβT and HbD are much lower than the normal ranges compared to HbS. The mean MCHC is much lower in HbβT, HbDD and HbS than the normal range. The cumulative gene frequency of haemoglobinopathies in India is 4.2%. With a population of over 1 billion and a birth rate of 28 per 1000, there are over 42 million carriers and over 12,000 infants are born each year with a major and clinical significant haemoglobinopathy. Out of these, clinically significant sickle cell anaemia and β-thalassaemic disorders account for almost equal numbers.

Conflicts of Interest

The authors declare no conflicts of interest.

Cite this paper

B. Urade, "Incidence of Sickle Cell Anaemia and Thalassaemia in Central India," Open Journal of Blood Diseases, Vol. 2 No. 4, 2012, pp. 71-80. doi: 10.4236/ojbd.2012.24014.

References

[1] H. Lehman and M. Cutbush, “Sickle Cell Trait in Southern India,” British Medical Journal, Vol. 1, No. 4755, 1952, pp. 404-405. HUdoi:10.1136/bmj.1.4755.404U
[2] R. S. Balgir, “The Burden of Haemoglobinopathies in India and the Challenges Ahead,” Current Science, Vol. 79, 2000, pp. 1536-1547.
[3] R. S. Balgir, “The Genetic Burden of Haemoglobinopathies with Special Reference to Community Health in India and the Challenges Ahead,” Indian Journal of Hematology and Blood Transfusion, Vol. 20, 2002, pp. 2-7.
[4] B. N. Shukla and B. R. Solanki, “Sickle Cell Anaemia in Central India,” The Lancet, Vol. 271, No. 7015, 1958, p. 297. HUdoi:10.1016/S0140-6736(58)91035-3U
[5] V. V. Deshmukh, “Deficiency of Erythrocyte Glucose-6Phosphate Dehydrogenase and Sickle-Cell Trait: A Survey at Aurangabad, Maharashtra,” Indian Journal of Medical Research, Vol. 56, No. 6, 1968, pp. 821-825.
[6] L. A. Ghatge, “Haemoglobins in Kurmi, Pradhan, PK Community of Madhya Pradesh,” Journal of the Medical Research, Vol. 66, No. 2, 1977, p. 260.
[7] R. S. Negi, “Population Dynamics of Sickle Cell Traits Distribution in India,” Ph.D. Thesis (Unpublished), University of Calcutta, Calcutta, 1976.
[8] S. L. Kate, “Health Problem of Tribal Population Groups from the State of Maharashtra,” Indian Journal of Medical Science, Vol. 55, No. 2, 2001, pp. 99-108.
[9] N. M. Blake, A. Ramesh, M. Vijaykumar, J. S. Murthy and K. K. Bhatia, “Genetic Studies on Some Tribes of the Telangana Region, Andhra Pradesh, India,” Acta Anthropogenetica, Vol. 5, No. 1, 1981, pp. 41-56.
[10] S. R. Das, N. Kumar, P. N. Bhattacharjee and D. B. Sastry, “Blood Groups (ABO, MN, & Rh), ABH Secretion, Sickle Cell, PTC Taste and Colour Blindness in the Mahar of Nagpur,” Journal of the Royal Anthropological Institute of Great Britain and Ireland, Vol. 91, No. 2, 1961, pp. 345-355. HUdoi:10.2307/2844419U
[11] H. P. Banker, S. L. Kate, G. D. Mokanshi, V. A. Kheedkar and M. A. Phadke, “Distribution of Sickle Cell Haemoglobin among Different Tribal Groups in Maharashtra State,” Indian Journal of Haematology, Vol. 2, 1984, p. 4.
[12] J. D. Goud and P. R. Rao, “Genetic Studies among the Five Tribal Populations of Andhra Pradesh, South India,” Anthropologischer Anzeiger, Vol. 37, No. 1, 1979, pp. 1-9.
[13] M. Jain, K. Das, P. B. S. V. Padmanabham, P. Dhar and V. R. Rao, “A1A2BO, Rh (D) Blood Groups and Haemoglobinopathies among Neo-Buddhist (Mahar) of Nagpur City,” Man in India, Vol. 84, No. 1-2, 2003, pp. 77-83.
[14] B. P. Urade, “Haemoglobinopathies in Vidarbha Region of Maharashtra,” National Conference on Prevention of Beta-Thalassaemia in India, Anthropological Survey of India, Kolkata, 7-9 March 2008.
[15] B. P. Urade and S. K. Mallick, “Incidence of Sickle Cell Anaemia in Chhattisgarh—A Review,” National Seminar, School of Studies in Anthropology, Pt. R.S.S. University, Raipur, 2003.
[16] B. P. Urade and S. K. Mallick, “Incidence of Sickle Cell Anaemia among the Mahar of Raipur, Chhattisgarh,” National Seminar, School of Studies in Anthropology, Pt. R.S.S. University, Raipur, 25-27 February 2004.
[17] B. P. Urade and M. Chakravarty, “Haemoglobinopathies of Central and North-Eastern India,” National Seminar, School of Studies in Anthropology, Pt. R.S.S. University, Raipur, 28-30 November 2006.
[18] B. P. Urade, M. Chakravarty and S. K. Mallick, “Sickle Cell Anaemia—A Genetically Handicap Disease,” In: R. K. Pathak, A. K. Sinha, B. G. Banerjee, R. N. Vasishat and C. J. Edwin, Eds., Bio-Social Issues in Health, Northern Book Centre, New Delhi, 2008, pp. 47-55.
[19] B. P. Urade, J. Verma and V. R. Rao, “Haemoglobinopathies in Maharashtra—Current Scenario and Challenges Ahead,” National Seminar on Human Genomic and Cultural Diversity at School of Studies in Anthropology, Pt. R.S.S. University, Raipur, 2-4 March 2009.
[20] G. Chakravarty and B. P. Urade, “Haematological Profile in Sickle Cell Anaemia and Thalassaemia Patients in Vidarbha Region of Maharashtra,” National Seminar on Human Genomic and Cultural Diversity at School of Studies in Anthropology, Pt. R.S.S. University, Raipur, 2-4 March 2009.
[21] J. K. Siddoo, S. K. Siddoo, W. H. Chase, L. Morgan Dean and W. H. Perry, “Thalassaemia in Sikhs,” Blood, Vol. 11, No. 3, 1956, pp. 197-210.
[22] R. S. Balgir, “Genetic Epidemiology of the Three Predominant Abnormal Haemoglobins in India,” Journal of the Associations of Physicians of India, Vol. 44, No. 1, 1996, pp. 5-28.
[23] A. Jawahirani, M. Mamtani, K. Das, V. Rughwani and H. Kulkarni, “Prevalence of β-Thalassaemia in Subcastes of Indian Sindhi—Results from a Two Phase Survey,” Public Health, Vol. 121, No. 3, 2007, pp. 193-198. HUdoi:10.1016/j.puhe.2006.10.017U
[24] N. Saha, B. Banerjee, “Incidence of Abnormal Haemoglobins in Punjab,” Calcutta Medical Journal, Vol. 62, 1965, pp. 82-86.
[25] J. V. Dacie and S. M. Lewis, “Practical Haematology,” Churchill Livingstone, Edinburgh, 1977.
[26] K. Betke, H. R. Marti and I. Schlicht, “Estimation of a Small Percentage of Foetal Haemoglobin,” Nature, Vol. 184, 1959, pp. 1877-1878, HUdoi:10.1038/1841877a0U
[27] K. G. Beutler, J. C. Kaplan, G. W. Lohr, B. Ramot, W. N. Valentine, “International Committee for Standardization in Haematology; Recommended Screening Test G6PD Deficiency,” British Journal of Haematology, Vol. 43, No. 3, 1979, pp. 43-46.
[28] R. S. Balgir, “Scenario of Haemoglobin Variants in Central-East Coast of India,” Current Science, Vol. 90, No. 12, 2006, pp. 1651-1657.
[29] S. Sinha, A. Kumar, V. Gupta, S. Kumar, V. P. Singh and R. Raman, “Haemoglobinopathies-Thalassaemias and Abnormal Haemoglobins in Eastern Uttar Pradesh and Adjoining Districts of Neighbouring States,” Current Science, Vol. 87, No. 6, 2004, pp. 775-780.
[30] M. K. Bhasin, H. Walter and H. Danker-Hopfe, “Glucose6-Phosphate Dehydrogenase Deficiency and Abnormal Haemoglobins (S and E) in People of India,” Journal of Human Ecology, Vol. 3, 1994, pp. 131-159.
[31] M. Vijaykumar, K. C. Malhotra, H. Walter, K. Gilbert, P. Landenberg, A. Dannewitz, A. Sorensen, R. Chakraborty, A. P. Reddy and B. N. Mukherjee, “Genetic Studies among the Siddis of Karnataka, India. A Migrant Population from Africa,” Zeitschrift fur Morphologie und Anthropologie, Vol. 77, No. 2, 1987, pp. 97-121.
[32] A. C. Gorakshakar, M. S. Sathe, S. R. Shirsat and H. M. Bhatia, “Genetic Studies in Ratnagiri and Sindhudurg Districts of Maharashtra—Incidence of ABO, Rh (D), Ina Antigens, G-6PD Deficiency and Abnormal Haemoglobins,” Journal of the Indian Anthropological Society, Vol. 22, 1987, pp. 38-46.
[33] S. M. A. Hakim, A. J. Baxi, V. Balkrishnan, K. V. Kulkarni, S. S. Rao and H. T. Jhala, “Haptoglobin, Transferrin and Abnormal Haemoglobin in Indian Muslims,” Indian Journal of Medical Research, Vol. 60, No. 5, 1972, pp. 699-703.
[34] N. R. Saha, L. Kirk, S. Shanbhag, S. R. Joshi and H. M. Bhatia, “Population Genetic Studies in Kerala and the Nilgiris (South West Asia),” Human Heredity, Vol. 26, 1976, pp.175-197. HUdoi:10.1159/000152802U
[35] V. R. Rao and H. M. Bhatia, “Quantitative Assessment of HbS in Sickle Cell Heterozygotes among Some Tribes in Maharashtra,” Indian Journal of Medical Research, Vol. 87, 1988, pp. 257-261.
[36] M. B. Agarwal, “The Burden of Haemoglobinopaties in India—Time to Wake Up?” Journal of the Associations of Physicians of India, Vol. 53, 2005, pp. 1017-1018.
[37] R. V. Russel and H. Lal, “Tribes and Castes of the Central Provinces of India,” Vol. 4, McMillon Company, London, 1916.
[38] G. M. Brittenham, “Globin Gene Variant and Polymorphism in India,” In: W. P. Winter, Ed., Haemoglobin Variants in Human Populations, CRC Press Inc., Florida, Vol. 2, 1988, pp. 79-109.
[39] C. S. Iswad and S. N. Naik, In: H. M. Bhatia and V. R. Rao, Eds., Genetic Atlas and Indian Tribes, ICMR, Bomba, 1986.
[40] F. B. Livingstone, “Who Gave Whom Haemoglobin S— The Use of Restriction Cite Haplotype Variation for the Interpretation of the Evolution of the βS Globin Gene,” American Journal of Human Biology, Vol. 1, No. 3, 1989, pp. 289-302. HUdoi:10.1002/ajhb.1310010309U
[41] D. R. Higgs, B. E. Aldridge, J. Lamb, J. B. Clegg, D. J. Weatherall, R. H. Hayes, Y. Grandison, Y. Lowrie, K. P. Mason, B. E. Searjeant and G. R. Searjeant, “The Interaction of Alpha Thalassaemia and Homozygous Sickle Cell Disease,” The New England Journal of Medicine, Vol. 306, No. 24, 1982, pp. 1441. HUdoi:10.1056/NEJM198206173062402U
[42] A. E. Kulozik, B. C. Kar, R. K. Satapathy, B. E. Searjeant, G. R. Searjeant and D. J. Weatherall, “Foetal Haemoglobin Levels and βS Globin Haplotypes in an Indian Population with Sickle Cell Disease,” Blood, Vol. 69, 1987, p. 1742.
[43] G. W. G. Bird and H. Lehmann, “Haemoglobin D in India,” British Medical Journal, Vol. 1, 1956, p. 514. HUdoi:10.1136/bmj.1.4965.514U
[44] I. Karve and V. M. Dandekar, “Anthropometric Measurement of Maharashtra, Deccan College Monograph,” Series No. 8, 1951.

  
comments powered by Disqus

Copyright © 2018 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.