Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion

Background: There is no report of hematological data in patients with co-inheritance of compound heterozygous hemoglobin (Hb) Hope/E and α-thalassemia-1 Southeast Asian (SEA) type deletion. Methods: Hematological parameters were studied in 3 Thai patients with compound heterozygous Hb Hope/E and 1 o...

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Main Author: Sakorn Pornprasert
Format: Journal
Published: 2018
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http://cmuir.cmu.ac.th/jspui/handle/6653943832/52199
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spelling th-cmuir.6653943832-521992018-09-04T09:22:00Z Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion Sakorn Pornprasert Biochemistry, Genetics and Molecular Biology Background: There is no report of hematological data in patients with co-inheritance of compound heterozygous hemoglobin (Hb) Hope/E and α-thalassemia-1 Southeast Asian (SEA) type deletion. Methods: Hematological parameters were studied in 3 Thai patients with compound heterozygous Hb Hope/E and 1 of them had α-thalassemia-1 SEA type deletion. Red cell counts, hemoglobin, hematocrit, and red cell indices were measured using an automated blood counter. The molecular analysis of Hb Hope was performed using the amplification refractory mutation system (ARMS)-PCR. Results: The lowest levels of hemoglobin, hematocrit, MCV, MCH, and HbA 2/E and the highest level of RDW were found in a patient who had co-inheritance of compound heterozygous Hb Hope/E and a-thalassemia-1 SEA type deletion. Conclusions: Co-inheritance of compound heterozygous Hb Hope/E and α-thalassemia-1 SEA type deletion caused prominent changes in hematological features. Thus, knowledge and understanding of these multiple gene interactions will assist in diagnosis, management, and counseling in these patients. 2018-09-04T09:22:00Z 2018-09-04T09:22:00Z 2013-10-30 Journal 14336510 2-s2.0-84886397629 10.7754/Clin.Lab.2013.130139 https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=84886397629&origin=inward http://cmuir.cmu.ac.th/jspui/handle/6653943832/52199
institution Chiang Mai University
building Chiang Mai University Library
country Thailand
collection CMU Intellectual Repository
topic Biochemistry, Genetics and Molecular Biology
spellingShingle Biochemistry, Genetics and Molecular Biology
Sakorn Pornprasert
Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
description Background: There is no report of hematological data in patients with co-inheritance of compound heterozygous hemoglobin (Hb) Hope/E and α-thalassemia-1 Southeast Asian (SEA) type deletion. Methods: Hematological parameters were studied in 3 Thai patients with compound heterozygous Hb Hope/E and 1 of them had α-thalassemia-1 SEA type deletion. Red cell counts, hemoglobin, hematocrit, and red cell indices were measured using an automated blood counter. The molecular analysis of Hb Hope was performed using the amplification refractory mutation system (ARMS)-PCR. Results: The lowest levels of hemoglobin, hematocrit, MCV, MCH, and HbA 2/E and the highest level of RDW were found in a patient who had co-inheritance of compound heterozygous Hb Hope/E and a-thalassemia-1 SEA type deletion. Conclusions: Co-inheritance of compound heterozygous Hb Hope/E and α-thalassemia-1 SEA type deletion caused prominent changes in hematological features. Thus, knowledge and understanding of these multiple gene interactions will assist in diagnosis, management, and counseling in these patients.
format Journal
author Sakorn Pornprasert
author_facet Sakorn Pornprasert
author_sort Sakorn Pornprasert
title Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
title_short Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
title_full Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
title_fullStr Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
title_full_unstemmed Hematological characterization of compound heterozygous hemoglobin Hope/E patients with and without α-Thalassemia-1 SEA type deletion
title_sort hematological characterization of compound heterozygous hemoglobin hope/e patients with and without α-thalassemia-1 sea type deletion
publishDate 2018
url https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=84886397629&origin=inward
http://cmuir.cmu.ac.th/jspui/handle/6653943832/52199
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