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Publication
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| Research Title |
Hb Hope [b136(H4)Gly-Asp] related disorders in Thailand: origin, interactions with various hemoglobinopathies and diagnostic aspects |
| Date of Distribution |
13 October 2010 |
| Conference |
| Title of the Conference |
XXXIII World Congress of the International Society of Hematology |
| Organiser |
Hematology conference ISH 2010, Kenes International |
| Conference Place |
ICC Jerusalem International Convention Center, 1 Shazar, Jerusalem, Israel |
| Province/State |
Jerusalem, Israel |
| Conference Date |
10 October 2010 |
| To |
13 October 2010 |
| Proceeding Paper |
| Volume |
- |
| Issue |
- |
| Page |
- |
| Editors/edition/publisher |
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| Abstract |
We described the molecular and hematological profiles associated with syndromes caused by interactions of Hb Hope with various hemoglobinopathies in 41 Thai patients. Nine different genotypes were observed which were classified into 4 groups. Group I included 25 Hb Hope heterozygotes with relatively normal hematological features and an average level of 42.2 % Hb Hope. Group II included those with Hb Hope and a-thalassemias including 1 double heterozygous Hb Hope/a-thalassemia 2, 1 Hb Hope/Hb Constant Spring, 3 Hb Hope/a-thalassemia 1 and 1 Hb Hope/Hb H disease. The average levels of Hb Hope were 48.5 %, 43.1 %, 14.3-29.6 % and 21.6 %, respectively. Minute amounts of Hb Bart’s but not Hb H was observed in a patient with Hb Hope/Hb H-disease. Group III included two compound Hb Hope/b0-thalassemia, 1 Hb Hope/b0-thalassemia/a-thalassemia 2 and 1 Hb Hope/b0-thalassemia/a-thalassemia 1. In this group, the average levels of Hb Hope were 90.1 %, 73.1 % and 78.5 %, respectively. Hb A2 was elevated in all cases. Group IV included 6 compound Hb Hope/Hb E heterozygotes with the average levels of 66.1 % Hb Hope and 27.8 % Hb E. Haplotype analysis demonstrated that all these Thai bHope genes were associated with the same haplotype, (+ - - - - + +), indicating likely a single origin of this variant in Thai population. Although Hb Hope is identified on both HPLC and capillary electrophoresis and clinically innocuous, differentiation from those of clinically relevant Hb variants would require DNA-based diagnostics. |
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| Peer Review Status |
ไม่มีผู้ประเมินอิสระ |
| Level of Conference |
นานาชาติ |
| Type of Proceeding |
Abstract |
| Type of Presentation |
Poster |
| Part of thesis |
true |
| Presentation awarding |
false |
| Attach file |
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| Citation |
0
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Journal Publication
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