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Suthat Fucharoen - One of the best experts on this subject based on the ideXlab platform.
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association oF snp in exon 1 oF hbs1l with Hemoglobin F level in β0 thalassemia Hemoglobin e
International Journal of Hematology, 2008Co-Authors: Riyaz Ahmad Pandit, Pranee Winichagoon, Suthat Fucharoen, Saovaros Svasti, Orapan Sripichai, Thongperm Munkongdee, Kanokporn Triwitayakorn, Chayanon PeerapittayamongkolAbstract:Increase in Fetal Hemoglobin (Hb F) reduces globin chain imbalance in β-thalassemia, consequently improving symptoms. QTL mapping together with previous genome-wide association study involving approximately 110,000 gene-based SNPs in mild and severe β0-thalassemia/Hb E patients revealed SNPs in HBS1L signiFicantly associated with severity and Hb F levels. Given its potential as binding site For transcription Factor activator protein 4, HBS1L exon 1 C32T polymorphism was genotyped in 455 cases, providing For the First time evidence that C allele is associated with elevated Hb F level among β0-thalassemia/Hb E patients with XmnI-Gγ−/−and XmnI-Gγ+/−polymorphisms.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a δ-globin Fragment showed a 27-kb deletion oF DNA that included the β-globin gene and the 3′ deoxyribonuclease I hypersensitive site 1 (3′HS1) sequence downstream. Deletion oF the insulator, 3′HS1, and the juxtaposition oF the HPFH-3 core enhancer downstream to the 3′ breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Aγ-globin gene (pEBAC/148β:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148β:EGFP construct with the HPFH-3 core enhancer immediately 5′ to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148β:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3′HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3′HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the γ-globin gene.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a delta-globin Fragment showed a 27-kb deletion oF DNA that included the beta-globin gene and the 3' deoxyribonuclease I hypersensitive site 1 (3'HS1) sequence downstream. Deletion oF the insulator, 3'HS1, and the juxta-position oF the HPFH-3 core enhancer downstream to the 3' breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Agamma-globin gene (pEBAC/148beta:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148beta:EGFP construct with the HPFH-3 core enhancer immediately 5' to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148beta:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3'HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3'HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the delta-globin gene.
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hydroxyurea increases Hemoglobin F levels and improves the eFFectiveness oF erythropoiesis in beta thalassemia Hemoglobin e disease
Blood, 1996Co-Authors: Suthat Fucharoen, Noppadol Siritanaratkul, P Winichagoon, Jew Chowthaworn, Wipapun Siriboon, Wanna Muangsup, Suchet Chaicharoen, Nalinee Poolsup, Busaba Chindavijak, P PootrakulAbstract:Hydroxyurea (HU) is one oF several agents that have been shown to enhance Hemoglobin (Hb) F levels in patients with sickle cell disease and may be useFul as a therapy For beta-globinopathies. However, limited inFormation exists on the eFFects oF HU in patients with thalassemia. Accordingly, we examined the hematologic eFFects oF orally administered HU in 13 patients with beta-thalassemia/Hb E, including Four patients who had been splenectomized. These patients were treated with escalating doses (Final range, 10 to 20 mg/kg/d) For 5 months and were observed in the outpatient hematology clinic every 2 to 4 weeks. Complete blood counts including reticulocyte counts, amounts oF Hb E and Hb F, G gamma:A gamma and alpha:non-alpha globin biosynthetic ratios were evaluated beFore and during treatment. Almost all patients responded with an average increase oF 33% in Hb F levels, From a mean (+/- SD) oF 42% +/- 11% to 56% +/- 8% (P < .0001), and a reciprocal decline in the percentage oF Hb E From 59% +/- 9% to 49% +/- 8% (P < .001). Reticulocytosis was decreased From a mean (+/- SD) oF 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a slight (10%) but statistically signiFicant increase in Hemoglobin levels and an improved balance in alpha:non-alpha globin chains ratios. The side eFFects were minimal in most patients, although these patients tended to tolerate a lower dose oF HU beFore signiFicant myelosuppression than has been our previous experience in sickle cell disease. One splenectomized patient died oF sepsis during the trial. We conclude that increased Hb F production in beta-thalassemia/Hb E patients, with an improvement in the alpha:non-alpha globin ratios and, probably, the eFFectiveness oF erythropoiesis, can be achieved using HU. Longer trials oF HU in this population, including at other doses and in combination with other agents, appear warranted.
Linda Smith M Resar - One of the best experts on this subject based on the ideXlab platform.
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pulsed dosing with oral sodium phenylbutyrate increases Hemoglobin F in a patient with sickle cell anemia
Pediatric Blood & Cancer, 2008Co-Authors: Patrick Hines, George J Dover, Linda Smith M ResarAbstract:Increasing Hemoglobin F (HbF) appears to be beneFicial For patients with sickle cell anemia. We previously demonstrated that daily, oral sodium phenylbutyrate (OSPB) induces HbF synthesis in pediatric and adult patients with Hemoglobin SS (HbSS). The high doses and need For daily therapy, however, have limited its use. Here, we report a patient treated with pulsed-dosing oF OSPB For over 3 years. This patient developed a modest, but sustained elevation in HbF over the course oF therapy without side eFFects. Although larger studies are needed, this case demonstrates that pulsed-dosing with OSPB enhances HbF synthesis. Pediatr Blood Cancer 2008;50:357–359. © 2007 Wiley-Liss, Inc.
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induction oF Fetal Hemoglobin synthesis in children with sickle cell anemia on low dose oral sodium phenylbutyrate therapy
Journal of Pediatric Hematology Oncology, 2002Co-Authors: Linda Smith M Resar, Lorna K Fitzpatric, Saul W Brusilow, Jodi B Segal, Alison M Friedmann, George J DoverAbstract:This study was designed to determine iF low doses oF oral sodium phenylbutyrate (SPB) induce Hemoglobin F (HbF) synthesis in children with Hemoglobin SS (HbSS). We treated 8 children with HbSS over a period oF 5-30 weeks. The initial dose (1.0 g/d) was increased weekly (by 1.0 g/d) until F-reticulocytes doubled. All patients showed an increase in F-reticulocytes (P = 0.002) that was dose-dependent (P = 0.001). Three oF 5 patients who continued oral SPB For more than 10 weeks had substantial increases in HbF. We conclude that lower dose SPB is eFFective in inducing HbF synthesis in some children with HbSS. Further trials are warranted to determine the optimal treatment regimen.
Pranee Winichagoon - One of the best experts on this subject based on the ideXlab platform.
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association oF snp in exon 1 oF hbs1l with Hemoglobin F level in β0 thalassemia Hemoglobin e
International Journal of Hematology, 2008Co-Authors: Riyaz Ahmad Pandit, Pranee Winichagoon, Suthat Fucharoen, Saovaros Svasti, Orapan Sripichai, Thongperm Munkongdee, Kanokporn Triwitayakorn, Chayanon PeerapittayamongkolAbstract:Increase in Fetal Hemoglobin (Hb F) reduces globin chain imbalance in β-thalassemia, consequently improving symptoms. QTL mapping together with previous genome-wide association study involving approximately 110,000 gene-based SNPs in mild and severe β0-thalassemia/Hb E patients revealed SNPs in HBS1L signiFicantly associated with severity and Hb F levels. Given its potential as binding site For transcription Factor activator protein 4, HBS1L exon 1 C32T polymorphism was genotyped in 455 cases, providing For the First time evidence that C allele is associated with elevated Hb F level among β0-thalassemia/Hb E patients with XmnI-Gγ−/−and XmnI-Gγ+/−polymorphisms.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a δ-globin Fragment showed a 27-kb deletion oF DNA that included the β-globin gene and the 3′ deoxyribonuclease I hypersensitive site 1 (3′HS1) sequence downstream. Deletion oF the insulator, 3′HS1, and the juxtaposition oF the HPFH-3 core enhancer downstream to the 3′ breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Aγ-globin gene (pEBAC/148β:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148β:EGFP construct with the HPFH-3 core enhancer immediately 5′ to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148β:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3′HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3′HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the γ-globin gene.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a delta-globin Fragment showed a 27-kb deletion oF DNA that included the beta-globin gene and the 3' deoxyribonuclease I hypersensitive site 1 (3'HS1) sequence downstream. Deletion oF the insulator, 3'HS1, and the juxta-position oF the HPFH-3 core enhancer downstream to the 3' breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Agamma-globin gene (pEBAC/148beta:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148beta:EGFP construct with the HPFH-3 core enhancer immediately 5' to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148beta:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3'HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3'HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the delta-globin gene.
Lillian C Mcmahon - One of the best experts on this subject based on the ideXlab platform.
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enhanced Hemoglobin F production in an eight year old with sickle cell disease receiving weekly iv methotrexate and daily oral 6 mercaptopurine For acute lymphoblastic leukemia 764
Pediatric Research, 1998Co-Authors: Eric F Grabowski, David H Ebb, Lillian C McmahonAbstract:Enhanced Hemoglobin F Production in an Eight-Year Old With Sickle Cell Disease Receiving Weekly IV Methotrexate and Daily Oral 6-Mercaptopurine For Acute Lymphoblastic Leukemia † 764
Khaimuk Changsri - One of the best experts on this subject based on the ideXlab platform.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a δ-globin Fragment showed a 27-kb deletion oF DNA that included the β-globin gene and the 3′ deoxyribonuclease I hypersensitive site 1 (3′HS1) sequence downstream. Deletion oF the insulator, 3′HS1, and the juxtaposition oF the HPFH-3 core enhancer downstream to the 3′ breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Aγ-globin gene (pEBAC/148β:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148β:EGFP construct with the HPFH-3 core enhancer immediately 5′ to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148β:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3′HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3′HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the γ-globin gene.
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molecular mechanism oF high Hemoglobin F production in southeast asian type hereditary persistence oF Fetal Hemoglobin
International Journal of Hematology, 2006Co-Authors: Khaimuk Changsri, Varaporn Akkarapathumwong, Duangporn Jamsai, Pranee Winichagoon, Suthat FucharoenAbstract:Hereditary persistence oF Fetal Hemoglobin (HPFH) is associated with a high level oF Hemoglobin F (HbF) synthesis in adult heterozygotes. In this study, 2 oF 6 unrelated HPFH Thai Families were Found to be Southeast Asian-type HPFH (SEA-HPFH) by analyses oF the hematologic data and Southern blot hybridization with polymerase chain reaction-ampliFied DNA probes. DNA mapping with a probe For a delta-globin Fragment showed a 27-kb deletion oF DNA that included the beta-globin gene and the 3' deoxyribonuclease I hypersensitive site 1 (3'HS1) sequence downstream. Deletion oF the insulator, 3'HS1, and the juxta-position oF the HPFH-3 core enhancer downstream to the 3' breakpoint have been postulated to be the cause oF high HbF production in these individuals. To test this hypothesis, we transFected K562 cells with 4 diFFerent bacterial artiFicial chromosome constructs containing the enhanced green Fluorescent protein (EGFP) gene at the position oF the Agamma-globin gene (pEBAC/148beta:EGFP). Flow cytometry was used to compare EGFP expression From the pEBAC/148beta:EGFP construct with the HPFH-3 core enhancer immediately 5' to the SEA-HPFH breakpoint (pEnH), From the pEBAC/148beta:EGFP construct with 8 kb oF the breakpoint sequence and the HPFH-3 core enhancer (pSEA-HPFH), and From the construct with 3'HS1 Followed by the pSEA-HPFH sequence (pSEA-HPFH_3pHS1). The results show that high HbF production in SEA-HPFH occurs From a deletion oF the 3'HS1 sequence and the juxtaposition oF the HPFH-3 enhancer downstream to the delta-globin gene.