Incidence of Sickle Cell Anaemia and Thalassaemia in Central India

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 (β T ) 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, Hbβ T , HbE and HbD. Examination of 6463 individuals showed high incidences for haemoglobin variants, HbS and Hbβ T 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 Hbβ T 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 HbA 2 . Unusual greater mean RBC and WBC suggest the high concentration of hypochromic microcytosis in anaemia. The mean MCV and MCH in Hbβ T and HbD are much lower than the normal ranges compared to HbS. The mean MCHC is much lower in Hbβ T , 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.

Premarital screening of college students for carrier detection in thalassemia and sickle cell disease: need of the hour

“…These hemoglobinopathies are a serious public health problem in central India, reflecting genetic heterogeneity of population with burden on available medical facilities. [2] Population-based screening programs for the detection of thalassemia and sickle cell anemia are implemented in this region.…”

“…In present study, the prevalence of sickle cell trait was found to be 3.4%. Urade [2] reported the prevalence of sickle cell trait to be 5.6%. Bhukhanvala et al [12] found its prevalence to be 2.41%.…”

“…These genetic diseases are controlled by a single gene that is transmitted from parents to offspring from one generation to another. [2] World Health Organization figures estimate that 7% world population is carrier for hemoglobin disorders, leading to high degree of morbidity and mortality. [3] Thalassemia are a group of congenital anaemia that have defect of one or more of the globin subunits of human hemoglobin due to point mutation.…”

<i>In Vitro</i> Sickling Inhibitory Effects and Anti-Sickle Erythrocytes Hemolysis of <i>Dicliptera colorata</i> C. B. Clarke, <i>Euphorbia hirta</i> L. and <i>Sorghum bicolor</i> (L.) Moench

“…It affects worldwide more than 50 million people. Each year about 300,000 children are born with pathological hemoglobin of which 70% are affected by SCD [2]. Most of them die before the age of five years [3].…”

Spectrum of hemoglobinopathies by high performance liquid chromatography with special reference to role of HbA2 levels at tertiary care centre

“…Other studies also reported maximum report of cases of sickle cell disorder in mahar caste (11.08%) by Bhaskar et al and Ambekar S.S. et al reported maximum cases of beta thalassaemia amongst Navbuddhas. 8,9 In the present study splenomegaly was found most common in the patients of β-thalassemia major (83.33%) followed by sickle-Beta thalassemia (15 cases), sickle cell Anemia (2) and sickle cell trait (4).…”