Hemoglobin SE disease in Hatay, in the southern part of Turkey

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dc.contributor.authorAcipayam, Can
dc.contributor.authorOktay, Gonul
dc.contributor.authorIlhan, Gul
dc.contributor.authorCuruk, Mehmet Akif
dc.date.accessioned2024-09-18T20:26:39Z
dc.date.available2024-09-18T20:26:39Z
dc.date.issued2015
dc.departmentHatay Mustafa Kemal Ãœniversitesien_US
dc.description.abstractDouble 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 Al: 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 Cukurova 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.en_US
dc.description.sponsorshipHemoglobinopathy Center, Hatay Antakya Government Hospital, Turkeyen_US
dc.description.sponsorshipthis study was supported bythe Hemoglobinopathy Center, Hatay Antakya Government Hospital, Turkeyen_US
dc.identifier.doi10.4081/thal.2015.4597
dc.identifier.endpage5en_US
dc.identifier.issn2039-4357
dc.identifier.issn2039-4365
dc.identifier.issue1en_US
dc.identifier.startpage3en_US
dc.identifier.urihttps://doi.org/10.4081/thal.2015.4597
dc.identifier.urihttps://hdl.handle.net/20.500.12483/10463
dc.identifier.volume5en_US
dc.identifier.wosWOS:000366114600002en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.language.isoenen_US
dc.publisherMdpien_US
dc.relation.ispartofThalassemia Reportsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjecthemoglobin Sen_US
dc.subjecthemoglobin Een_US
dc.subjecthemoglobin SEen_US
dc.subjectvaso-occlusive crisisen_US
dc.subjectcerebral strokeen_US
dc.titleHemoglobin SE disease in Hatay, in the southern part of Turkeyen_US
dc.typeArticleen_US

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