Abstract:
:The present study aimed at differentiating rare types of heterozygous beta-thalassemia (thal) with normal Hb A(2) values from alpha-thal in Iranian carriers by globin chain synthesis in addition to other hematological parameters. Our study groups consisted of 51 normal subjects, 24 heterozygous beta- thalassemic subjects with high Hb A(2), 62 alpha-thal-2 subjects, 34 alpha-thal-1 subjects, six Hb H disease thalassemic subjects, 14 silent beta-thal subjects with normal Hb A(2) values, five deltabeta-thal subjects and two subjects with an association of alpha- and deltabeta-thal (total = 198). Analysis of globin chains was performed by high performance liquid chromatography (HPLC). The results showed that the alpha/beta ratio averages were close to the ones in the published literature, but with a greater standard deviation and a wider range. Globin chain synthesis (GCS) could be valuable in differentiating between microcytosis produced by silent beta-thal (heterozygous beta-thal with a normal Hb A(2) level) and that caused by alpha-thal. Since the complex genotype/phenotype relationship can lead to diagnostic difficulties, GCS cannot be used as the only diagnostic tool for thalassemia carrier detection. Therefore, a combination of different tests for each patient is required.
journal_name
Hemoglobinjournal_title
Hemoglobinauthors
Khatami S,Dehboneh SR,Sadeghi S,Mirzazadeh R,Saeedi P,Bayat P,Najmabadi H,Zeinali S,Akbari MT,Ardjmand M,Amirkhani Adoi
10.1080/03630260701462006subject
Has Abstractpub_date
2007-01-01 00:00:00pages
333-41issue
3eissn
0363-0269issn
1532-432Xpii
780669658journal_volume
31pub_type
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