Effect of transfusion, iron chelation and splenectomy therapies in HbE/β-thalassaemia individuals in Malaysia
Abstract
HbE/β-thalassaemia is a compound heterozygous mutation with a vast clinical phenotype [1]. To improve quality of life, HbE/β-thalassaemia individuals receive different treatment strategies, either individually or in combination with therapy(ies), including blood transfusion, iron chelation and splenectomy [2-3]. Thus far, there are limited studies conducted regarding the effect of treatments in HbE/β-thalassaemia individuals. We hereby investigated the effect of treatments with respect to red blood cell indices, haemoglobin subtypes and gene expressions among 30 HbE/beta-thalassaemia individuals. Statistical analyses were carried out using SPSS 17.0. As compared to single therapy (transfused only individuals) and double therapies (transfused-chelated only individuals), individuals receiving triple therapies (transfused-chelated-splenectomised individuals) showed significantly high mean cell volume (MCV), mean cell haemoglobin (MCH) and reticulocytes count (Fig.1).Â
These findings suggest that triple therapies are the most effective in ameliorating the severity of the disease in terms of microcytosis and hypochromia [3-5]. The high reticulocyte count in triple therapies also allows the bone marrow to actively produce red blood cells suggesting that these therapies have clinical benefits by suppressing the ineffective erythropoiesis and improving the erythropoietic environment significantly among HbE/β-thalassaemia individuals in our studied group [6-7].
The effectiveness of these treatments is different among each HbE/β-thalassaemia individual whereby clinical variabilities among them could be a contributing factor. Triple therapies giving the best advantage to the HbE/β-thalassaemia patient in this study.
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References
Olivieri, N. F., et al., Hb E/beta-thalassaemia: a common & clinically diverse disorder. The Indian journal of medical research, 2011. 134: p. 522-531.
Rachmilewitz, E. A., et al., How I treat thalassemia. Blood, 2011, 118(13): p. 3479-3488.
Galanello, R., et al., Beta-thalassemia. Orphanet Journal of Rare Diseases, 2010. 5(11).
Piga, A., et al., Changing patterns of splenectomy in transfusion-dependent thalassemia patients. American Journal of Hematology, 2011. 5(suppl4): p. 385-388.
Zhou, Ya-Li., et al., Splenectomy improves anaemia but does not reduce iron burden in patients with haemoglobin H Constant Spring disease. Blood Transfusion, 2014. 12: p. 471-478.
Cortellazzi, L. C., et al., Reticulocyte parameters in hemoglobinopathies and iron deficiency anemia. Revista Brasileira de Hematologia e Hemoterapia, 2003. 25(2): p. 97-102.
Wagner, S. C., et al., Reticulocytes indices in β thalassemia trait individuals. Revista Brasileira de Hematologia e Hemoterapia, 2011. 33(5): p. 393-397.
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