Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?

Background: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which d...

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Main Authors: Bhushan Warpe, AV Shrikhande, SV Poflee
Format: Article
Language:English
Published: Iranian Society of Pathology 2016-04-01
Series:Iranian Journal of Pathology
Subjects:
Online Access:https://ijp.iranpath.org/article_18957_0bcfdfdf0617b85acef6b075b4b8792d.pdf
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author Bhushan Warpe
AV Shrikhande
SV Poflee
author_facet Bhushan Warpe
AV Shrikhande
SV Poflee
author_sort Bhushan Warpe
collection DOAJ
description Background: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical course of the patients. This is a pioneer study conducted on Central Indian poor population to use the already proven six studies to screen associated alpha-thalassemia in SCT patients thus, circumventing the much costlier alpha-genotyping studies. Moreover, it aimed to study the haematological parameters in such cases. Methods: The study was performed at RHDMC, IGGMC, Nagpur, India from 2003 to 2012. The sample population was suspected cases of haemolytic anaemia. CBC and RBC indices were obtained by a cell analyzer. The sickle solubility test positively screened cases were confirmed by agar-gel haemoglobin electrophoresis at pH 8.6. Finally, quantitative assessment of haemoglobin variants was performed by HPLC. Results: Out of total 5819 cases over ten years, 933 cases were sickle heterozygotes. Overall, 180/933 subjects were predicted to be homozygous alpha-thalassemia and 338/933 were heterozygous alpha-thalassemia, based on trimodal distribution of HbS. Conclusion: Genotyping is costlier for majority of the poor non-affording patients in Indian government set-ups, so this study is suitable to screen for associated alpha-thalassemia in SCT patients.
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spelling doaj.art-b6e77ec0114f4d89aa8563b9f90f00f92022-12-21T19:21:28ZengIranian Society of PathologyIranian Journal of Pathology1735-53032345-36562016-04-0111213313718957Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?Bhushan Warpe0AV Shrikhande1SV Poflee2Regional Haemoglobinopathy Detection & Management Centre (RHDMC), Department of Pathology, IGGMCH, Nagpur City-Maharashtra State, IndiaRegional Haemoglobinopathy Detection & Management Centre (RHDMC), Department of Pathology, IGGMCH, Nagpur City-Maharashtra State, IndiaRegional Haemoglobinopathy Detection & Management Centre (RHDMC), Department of Pathology, IGGMCH, Nagpur City-Maharashtra State, IndiaBackground: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical course of the patients. This is a pioneer study conducted on Central Indian poor population to use the already proven six studies to screen associated alpha-thalassemia in SCT patients thus, circumventing the much costlier alpha-genotyping studies. Moreover, it aimed to study the haematological parameters in such cases. Methods: The study was performed at RHDMC, IGGMC, Nagpur, India from 2003 to 2012. The sample population was suspected cases of haemolytic anaemia. CBC and RBC indices were obtained by a cell analyzer. The sickle solubility test positively screened cases were confirmed by agar-gel haemoglobin electrophoresis at pH 8.6. Finally, quantitative assessment of haemoglobin variants was performed by HPLC. Results: Out of total 5819 cases over ten years, 933 cases were sickle heterozygotes. Overall, 180/933 subjects were predicted to be homozygous alpha-thalassemia and 338/933 were heterozygous alpha-thalassemia, based on trimodal distribution of HbS. Conclusion: Genotyping is costlier for majority of the poor non-affording patients in Indian government set-ups, so this study is suitable to screen for associated alpha-thalassemia in SCT patients.https://ijp.iranpath.org/article_18957_0bcfdfdf0617b85acef6b075b4b8792d.pdftrimodal distributionhbsalpha-thalassaemiasickle cell traitmchmcvmchc
spellingShingle Bhushan Warpe
AV Shrikhande
SV Poflee
Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
Iranian Journal of Pathology
trimodal distribution
hbs
alpha-thalassaemia
sickle cell trait
mch
mcv
mchc
title Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
title_full Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
title_fullStr Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
title_full_unstemmed Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
title_short Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
title_sort can trimodal distribution of hbs levels in sickle cell traits be used to predict the associated alpha thalassemia for screening cases in central india
topic trimodal distribution
hbs
alpha-thalassaemia
sickle cell trait
mch
mcv
mchc
url https://ijp.iranpath.org/article_18957_0bcfdfdf0617b85acef6b075b4b8792d.pdf
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AT svpoflee cantrimodaldistributionofhbslevelsinsicklecelltraitsbeusedtopredicttheassociatedalphathalassemiaforscreeningcasesincentralindia