Main Article Content
A retrospective evaluation of the growth of the liver and spleen among 113 patients (66 males, 47 females) with transfusion dependent hemoglobin E/beta-thalassemia (Hb E/β thal) was reported. They received regular transfusions of leucocyte poor red blood cells 10-15 mL/kg every 4 weeks to maintain their pretransfusion Hb at 7-8 g/dL in group 1 (n = 25) and at least 9 g/dL in group 2 (n = 88). A healthy lifestyle involving diet, water intake, sleep, exercise and personal hygiene was advised. Iron chelation was provided when the serum ferritin level approached > 1,000 ng/mL. The results revealed that thalassemia facies were found in group 1 only. The Z-scores of weight and height and the final height of patients in group 1 did not significantly differ from those of group 2. However, the percentage of liver and spleen growth ≥ 5 cm below the costal margin in group 1 was significantly higher than those observed in group 2. Therefore, regular transfusion with pretransfusion Hb at least 9.0 g/dL and subsequent iron chelation should be adequately provided to transfusion dependent Hb E/β thal to achieve the optimal growth and the size of the liver and spleen, less than 5 cm below the costal margin.
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
2. Premawardhena A, De Silver S, Arambepola M, Olivieri NF, Vichinsky EP, Merson L, et al. Hemoglobin E-beta-thalassemia: Progress report from the International Study Group. Ann N Y Acad Sci. 2005;1054:33-9.
3. Fucharoen S, Winichagoon P, Pootrakul P, Piankijagum A, Wasi P. Variable severity of Southeast Asian beta 0-thalassemia/HbE disease. Birth Defects Orig Artic Ser. 1988;23:241-8.
4. Winichagoon P, Thonglairoam V, Fucharoen S, Wilairat P, Fukumaki Y, Wasi P. Severity differences in beta-thalassemia/ haemoglobin E syndromes: implication of genetic factors. Br J Haematol. 1993;83:633-9.
5. Mahachoklertwattana P, Yimsumruay T, Poomthavorn P, Chuansumrit A, Khlairit P. Acute effects of blood transfusion on growth hormone and insulin-like growth factor-1 levels in children with thalassemia. Horm Res Paediatr. 2011;75:240-5.
6. Viprakasit V. Comprehensive management for thalassemia. J Hematol Transf Med. 2013;23:303-20.
7. Sripichai O, Makarasara W, Munkongdee T, Kumkhaek C, Nuchprayoon I, Chuansumrit A, et al. A scoring system for the classification of β-thalassemia/Hb E disease severity. Am J Hematol. 2008;83:482-4.
8. Chapter 5: Iron Overload. In: Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, editors. Guidelines for the Clinical Management of Thalassemia: Thalassemia International Federation. Nicosia, Cyprus (CY): Thalassaemia International Federation; 2000.p. 21-35.
9. Trompeter S, Cohen A. Blood transfusion. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V, eds. Guidelines for the management of transfusion dependent thalassemia (TDT). 3rd ed. Nicosia (CY): Thalassemia International Federation; 2014. p. 28-41.
10. Songdej D, Sirachainan N, Wongwerawattanakoon P, Sasanakul W, Kadegasem P, Sungkarat W, et al. Combined chelation therapy with daily oral deferiprone and twice-weekly subcutaneous infusion of desferrioxamine in children with β-thalassemia: 3-year experience. Acta Haematol. 2015;133:226-36.
11. Shalitin S, Carmi D, Weintrob N, Phillip M, Miskin H, Kornreich L, et al. Serum ferritin level as a predictor of impaired growth and puberty in thalassemia major patients. Eur J Haematol. 2005;74:93-100.
12. Chuansumrit A, Songdej D, Sirachainan N, Wongwerawattanakoon P, Kadegasem P, Sasanakul W. Safety profile of a liquid formulation of deferiprone in young children with transfusion-induced iron overload: a 1-year experience. Paediatr Int Child Health 2016;36:209-13.
13. Schillie S, Murphy TV, Sawyer M, Ly K, Hughes E, Jiles R, et al. CDC guidance for evaluating health-care personnel for hepatitis B virus protection and for administering postexposure management. MMWR Recomm Rep. 2013;62(RR-10):1-19.