Hemoglobin SE Disease in Hatay, in the Southern Part of Turkey

Can Acipayam,Mehmet Akif Çürük,Gul Ilhan,Gonul Oktay

doi:10.4081/thal.2015.4597

Can Acipayam, Mehmet Akif Çürük + Show 2 more

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https://doi.org/10.4081/thal.2015.4597

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Journal: Thalassemia reports	Publication Date: Feb 24, 2015
Citations: 3	License type: CC BY 4.0

Affiliation: Cukurova University

Abstract

Double heterozygosity for hemoglobin (Hb) E and S, known as HbSE disease, is a rare, clinically benign condition involving mild hemolysis. Only 25 cases have been reported to date. The current literature generally associates HbSE with a benign clinical course, although vaso-occlusive complications have been reported. Although only single case reports were previously available, we have observed 20 cases of HbSE and wish to report them. We examined the records of patients presenting to our hemoglobinopathy center in 2001–2013. High performance liquid chromatography (HPLC) was used for hematological assessment of blood samples with ethylenediaminetetraacetic acid. Eight patients were male and 12 female. Mean hemoglobin electrophoresis values were Hb A1: 6.3%, Hb E 34.5%, HbS: 59.5% and Hb F: 1.9%. Three patients (15%) were symptomatic with vaso-occlusive crisis and one had cerebral stroke. These were siblings. The patient with cerebral stroke was using hydroxyurea. The incidence of HbSE disease is rising due to population admixtures and racial intermarriages. Increased numbers of cases of HbSE have been detected after premarital hemoglobinopathy screening in the Antakya and Çukurova regions of Turkey. The aim of this study was to report large numbers of patients with HbSE diagnosed through the routine HPLC method. The secondary aim was to emphasize that severe vaso-occlusive crisis such as infarction symptoms could be seen in HbSE.

Highlights

Materials and Methods ground noise is reduced with the use of a secondary wavelength at 690 nm.[10]
Population screening should ent of increasing ionic strength to the caremphasize that severe vaso-occlusive crisis be performed, and individuals with HbSE dis- tridge, where the hemoglobin fractions are such as infarction symptoms could be seen in ease should be identified and carefully moni- separated based on their ionic interaction with the cartridge material
mean cell volume (MCV) chest syndrome, painful episodes, splenic plus Hb E was suspected in those individuals was slightly below the normal range, 69.9±5.4 infarcts, hematuria, bone pain, avascular with very high hemoglobin A2 values, greater fL

Summary

Introduction

Materials and Methods ground noise is reduced with the use of a secondary wavelength at 690 nm.[10]. Three patients (15%) were symptomatic with vaso-occlusive crisis and n one had cerebral stroke. Population screening should ent of increasing ionic strength to the caremphasize that severe vaso-occlusive crisis be performed, and individuals with HbSE dis- tridge, where the hemoglobin fractions are such as infarction symptoms could be seen in ease should be identified and carefully moni- separated based on their ionic interaction with the cartridge material.

Results

Conclusion