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Open accessJournal ArticleDOI: 10.9790/0853-0955255

A study of Serological and Hematological Parameters in Thalassaemic Patients of Gujarat, Western India

01 Jan 2013-IOSR Journal of Dental and Medical Sciences-Vol. 9, Iss: 5, pp 52-55
Abstract: Absract: Thalassemia describes a group of inherited disorders characterized by reduced amount of hemoglobin, the oxygen-carrying protein inside the red blood cells. Various haematological and serological parameters were studied in a cohort of thalasaemic patients coming for routine blood transfusion regime in a government run children hospital of Rajkot. Due to poor socio-economic condition, none of the patients were taking chelation therapy needed for better management of thalassaemia. To get insight into effect of multiple blood transfusions on these patients, age, spleen status, distribution of blood group, hemoglobin level and other hematological indices and serolological parameter like SGPT related to iron overburden was studied. A very low hemoglobin level with very high SGPT level along with sleenomegaly is suggestive of poor prognosis in these patients.

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Topics: Thalassemia (59%), Blood transfusion (57%)
References
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Open accessJournal ArticleDOI: 10.1309/R1YL4B4BT2WCQDGV
Dinesh A. Rathod, Amarjeet Kaur, Vinod Patel, Kunjal Patel  +4 moreInstitutions (1)
Abstract: The present study aimed to retrospectively evaluate the usefulness of cell counter–based parameters and formulas in β-thalassemia trait (BTT) detection. The study included 170 BTT cases (hemoglobin [Hb]A 2 >4.0% [0.04]) and 30 non-BTT cases (HbA 2 , 2.3%3.5% [0.02-0.04]). Depending on the hemoglobin level and iron deficiency, the BTT group was further classified into classic BTT (n = 112) and BTT with iron deficiency anemia (n = 58). The RBC count, MCH, MCV, RDW, and Shine and Lal, Mentzler, Srivastava, England and Fraser, Ricerca, and Green indexes were applied. For the first time in the population of India, these 10 cell counter parameters and manual formulas were compared with high-performance liquid chromatography–derived HbA 2 levels for deriving a cost-effective alternative method; and receiver operating characteristic curves were applied. We found that the Shine and Lal, Srivastava, and Mentzler indexes, MCV, and MCH have better discriminative function than the RBC count and red cell distribution width and their related formulas. Thalassemia is a genetic disorder affecting synthesis of the globin moiety of the hemoglobin molecule. β-Thalassemia is prevalent in a broad belt extending from the Mediterranean basin to Southeast Asia. It is estimated that 1.5% of the world’s population carries β-thalassemia, ie, at least 80 to 90 million people with an estimated 60,000 new carriers born each year. The Southeast Asian region (which includes India, Thailand, and Indonesia) accounts for about 50% of the world’s carriers, ie, around 40 million people and almost half of homozygous births, whereas in the developed world, Europe and the Americas jointly account for just 10% to 13% of the world’s carriers. 1 The prevalence of β-thalassemia trait (BTT) is about 3.3% in India. In various parts of India, the prevalence of BTT is different: 6.5% in Punjab, 8.4% in Tamilnadu, 4.3% in south India, and 3.5% in Bengal. βThalassemia has a high prevalence in some communities, such as Sindhi, Luvana, Tribes, and Rajputs. The incidence of BTT in Gujarat is 10% to 15% in these communities, whereas the incidence in the general population is 2% to 3%. 2 Because a majority of people with BTT are asymptomatic, they may not be aware of their carrier state. BTT is associated with mild or no anemia but with reduced mean corpuscular volume (MCV) mean corpuscular hemoglobin (MCH) values and an elevated hemoglobin A 2 (HbA 2 ) level. The prevention of this homozygous condition can be

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Topics: Southeast asian (54%), Hemoglobin A2 (53%), Population (52%) ...read more

75 Citations


Journal ArticleDOI: 10.1111/J.1365-2141.1993.TB03346.X
Abstract: We have identified three individuals of Greek or Greek Cypriot origin with an atypical form of HbH disease characterized by a severe hypochromic microcytic anaemia associated with relatively small amounts of HbH in the peripheral blood Molecular analysis has shown that each is a compound heterozygote for a previously described mutation affecting the poly A addition signal (AATAAA-->AATAAG) and a previously undescribed mutation involving a T-->C transition in codon 29 of the alpha 2 gene causing a leucine-->proline substitution Although this mutation would be expected to produce an unstable haemoglobin and hence a haemolytic anaemia, simple heterozygotes for the alpha 29Leu-->Pro mutation have the phenotype of alpha-thalassaemia trait

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48 Citations



Open accessJournal ArticleDOI: 10.1043/1543-2165-132.11.1739
Abstract: Context.—Thalassemia and hemoglobinopathies are major public health problems worldwide. To establish a cost-effective screening tool for newborns in regions where the incidence of these disorders is significant, study of the hemoglobin and hematologic features of normal and thalassemic newborns is necessary. Objective.—To study hemoglobin and hematologic characteristics of normal and various thalassemic newborns and to assess the effectiveness of simple screening methods for α-thalassemia 1 and hemoglobin E. Design.—Study was made of 402 cord blood specimens collected from unrelated Thai individuals. Hematologic parameters and hemoglobin profiles were determined. Thalassemia mutations were identified using polymerase chain reaction–related techniques. Results.—As many as 178 subjects (44.3%) were found to carry thalassemia genes with 18 different genotypes. All forms of α-thalassemia including double heterozygote for hemoglobin E and α-thalassemia showed significant reduction in hemoglobin, mean ...

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Topics: Hemoglobin E (67%), Thalassemia (66%), Hemoglobin (53%)

20 Citations


Open accessJournal ArticleDOI: 10.1159/000048659
Abstract: Objectives: To analyze the results of hemoglobin electrophoresis (HE) in the routine laboratory of a tertiary hospital in Kuwait and to review the common types of hemoglobinopathies

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18 Citations