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Information Journal Paper

Title

IN VITRO HB PRODUCTION IN Β-THALASSEMIA PATIENTS IS NOT A PREDICTOR OF CLINICAL RESPONSIVENESS TO HYDROXYUREA

Pages

  948-956

Abstract

 Background: The hematologic response to HYDROXYUREA (HU) is varied among β-thalassemia (BT) patients. The BCL11A and SOX6 genes are involved in response to HU. This study aimed to investigate the in-vitro responsiveness of HU among BT major patients homozygote for IVSII-1G>A mutation and XmnI single nucleotide polymorphism (SNP) in order to find whether the in-vitro Hb concentration is a predictor of clinical (HU) responsiveness.Methods: In this case-control study, twenty BT patients homozygote for IVSII-1G>A mutation and XmnI SNP from THALASSEMIA Research Center, Sari, Iran in 2015 were selected and categorized into two groups of 10 Responder (R) and 10 Non-Responder (NR) according to their clinical HU response. Ten healthy individuals as a control group were also selected. Hematopoietic erythroid progenitors were expanded from peripheral blood. Hb concentration was measured using photometry method. The flow cytometry and real-time PCR methods were applied for the analysis of cell surface markers (CD71 and CD235a) and gene expression (BCL11A and SOX6), respectively.Results: R and NR groups produced higher amount of Basic Hb than C group in cell culture medium at day 14 (P<0.05). After HU treatment, in R group, Hb levels was significantly elevated in comparison to NR and C group (P<0.05). BCL11A expression was decreased after exposure to HU in all groups while SOX6 expression was only down-regulated in C group, and its expression was increased in R and NR groups after HU treatment.Conclusion: Since different factors including wide networks of intracellular factors and individual differences be-tween patients can affect response to HU in patients, the increasing HEMOGLOBIN on culture medium alone cannot predict clinical responsiveness to that drug.

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    APA: Copy

    MAHDAVI, MOHAMMAD REZA. (2017). IN VITRO HB PRODUCTION IN ?-THALASSEMIA PATIENTS IS NOT A PREDICTOR OF CLINICAL RESPONSIVENESS TO HYDROXYUREA. IRANIAN JOURNAL OF PUBLIC HEALTH, 46(7), 948-956. SID. https://sid.ir/paper/274194/en

    Vancouver: Copy

    MAHDAVI MOHAMMAD REZA. IN VITRO HB PRODUCTION IN ?-THALASSEMIA PATIENTS IS NOT A PREDICTOR OF CLINICAL RESPONSIVENESS TO HYDROXYUREA. IRANIAN JOURNAL OF PUBLIC HEALTH[Internet]. 2017;46(7):948-956. Available from: https://sid.ir/paper/274194/en

    IEEE: Copy

    MOHAMMAD REZA MAHDAVI, “IN VITRO HB PRODUCTION IN ?-THALASSEMIA PATIENTS IS NOT A PREDICTOR OF CLINICAL RESPONSIVENESS TO HYDROXYUREA,” IRANIAN JOURNAL OF PUBLIC HEALTH, vol. 46, no. 7, pp. 948–956, 2017, [Online]. Available: https://sid.ir/paper/274194/en

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