Response to hydroxyurea therapy in beta-thalassemia

American Journal of Hematology
Ariel KorenHannah Tamary

Abstract

Although a relatively small number of previous studies suggest a modest response to hydroxyurea (HU) therapy in beta-thalassemia, more recent investigations have revealed that some transfusion-dependent patients can become transfusion-independent following HU therapy. Patients with Ggamma XmnI polymorphism, several beta-globin mutations, and alpha-thalassemia deletions were inconsistently reported to have significant responses to HU therapy. To better predict who may respond, we retrospectively evaluated the clinical response and the molecular background of 18 beta-thalassemia patients treated with HU for a mean of 46 months. The majority of transfusion-dependent patients responded to HU therapy with 9 out of 11 (82%) becoming transfusion-independent. Five thalassemia intermedia (TI) patients receiving occasional blood transfusion did not require any additional transfusions following therapy and two TI patients who had never received transfusions had a 2 g/dl increase in their hemoglobin level. The majority of beta-thalassemia major patients who became transfusion-independent (7/9) were either homozygous (5) or heterozygous (2) for the XmnI polymorphism. No correlation was identified between response to therapy and the presence...Continue Reading

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Citations

Dec 20, 2011·International Journal of Hematology·Mehran KarimiMajid Yavarian
Feb 8, 2013·Journal of Pediatric Hematology/oncology·Saqib H AnsariMoinuddin Moinuddin
Apr 5, 2012·Cold Spring Harbor Perspectives in Medicine·Natasha Arora, George Q Daley
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Jan 31, 2018·International Journal of Hematology·Chingiz AsadovJeyhun Mammadov
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