Abstract:
:Three Indonesian patients with identical genotypes, each compound heterozygotes for Filipino beta(o)-thalassemia/HbE, expressed different clinical severities. One patient has mild disease and is transfusion independent, while the other two are severely affected and transfusion dependent. The size of the Filipino beta(o)-globin gene deletion was confirmed to be 45 kb, resolving conflicting values given in the literature. Neither ameliorating genetic factors such as alpha-globin gene deletions or the XmnI restriction site polymorphism at position -158 upstream of the (G)gamma-globin gene, nor differences in beta-globin gene haplotype, explain the phenotypic variation. These observations have implications for the development of antenatal diagnosis in Indonesia, as at present it is not possible to give an accurate prediction of severity of phenotype for this common genotype.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Setianingsih I,Williamson R,Daud D,Harahap A,Marzuki S,Forrest Sdoi
10.1002/(sici)1096-8652(199909)62:1<7::aid-ajh2>3.keywords:
subject
Has Abstractpub_date
1999-09-01 00:00:00pages
7-12issue
1eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199909)62:1<7::AID-AJH2>3.journal_volume
62pub_type
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