Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia

Pustika A. Wahidiyat, Sudigdo Sastroasmoro, Suthat Fucharoen, Iswari Setianingsih, Siti A. Putriasih



DOI: https://doi.org/10.13181/mji.v27i1.1779

Abstract


Background: β-thalassemia/HbE presents with a variety of clinical symptoms, from asymptomatic to severe, requiring routine transfusion. However, there is currently no agreed classification system to stratify patients based on clinical severity of β-thalassemia/HbE in the Indonesian population. Thailand has already established a classification system, and this study aimed to identify the applicability of the Thailand clinical scoring criteria to determine the severity of β-thalassemia/HbE in the Indonesian population.

Methods: This descriptive study was conducted by evaluating patients with β-thalassemia/HbE, who were classified into mild, moderate, or severe groups based on the Thailand clinical scoring criteria.

Results: A total of 293 subjects with β-thalassemia/HbE were included. Based on this clinical scoring criteria, it was found that only 21.5% of patients were classified as mild, and the remaining 35.5% and 44% were classified as moderate and severe respectively. Approximately 68.2% of the subjects in the severe group received transfusion at <4 years old, while only 10% of those in the mild group were transfused at the same age. In the mild group, only 10% of the subjects underwent routine transfusion, compared to 98.4% of the subjects in the severe group. In addition, only 27% of the subjects in the mild group showed stunted growth, while that in the moderate and severe groups were 54.5% and 86.8%, respectively.

Conclusion: Thailand clinical scoring criteria is able to determine the severity of Indonesia thalassemia patient which needs further management, i.e. transfusion and observation of stunted growth. This scoring system will help provide the provision of the most ideal management for the groups of patients based on their requirements.

Keywords


β-thalassemia/HbE; clinical criteria; Indonesia; scoring; Thailand

Full Text:

PDF

References


  1. Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;88(6):480–7. https://doi.org/10.2471/BLT.06.036673
  2. Fucharoen S, Weatherall DJ. The hemoglobin E thalassemias. Cold Spring Harb Perspect Med. 2012;2(8):a011734. https://doi.org/10.1101/cshperspect.a011734
  3. Danjou F, Anni F, Galanello R. Beta-thalassemia: from genotype to phenotype. Haematologica. 2011;96(11):1573–5. https://doi.org/10.3324/haematol.2011.055962
  4. Sripichai O, Makarasara W, Munkongdee T, Kumkhaek C, Nuchprayoon I, Chuansumrit A, et al. A scoring system for the classification of beta-thalassemia/HbE diasease severity. Am J Hematol. 2008;83(6):482–4. https://doi.org/10.1002/ajh.21130
  5. Soares P, Trejaut JA, Loo JH, Hill C, Mormina M, Lee CL, et al. Climate change and postglacial human dispersals in Southeast Asia. Mol Biol Evol. 2008;25(6):1209–18. https://doi.org/10.1093/molbev/msn068
  6. Wahidiyat PA. Thalassemia and its problems in Indonesia [Lecture]. Presented at: The 2nd international scientific meeting on hematology, oncology, thrombosis, and transplantation/transfusion in indonesia. Jakarta; 2016.
  7. Sahu PK, Pati SS, Mishra SK. Genotype-phenotype correlation of b-thalassemia spectrum of mutations in an Indian population. Hematol Rep. 2012;4(2):e9. https://doi.org/10.4081/hr.2012.e9
  8. Musallam KM, Taher AT, Rachmilewitz EA. b-Thalassemia intermedia: a clinical perspective. Cold Spring Harb Perspect Med. 2012;2(7):a013482. https://doi.org/10.1101/cshperspect.a013482
  9. Fucharoen S, Winichagoon P. Clinical and hematological aspects of hemoglobin E beta-thalassemia. Curr Opin Hematol. 2000;7(2):106–12. https://doi.org/10.1097/00062752-200003000-00006
  10. Panigrahi I, Agarwal S. Genetic determinants of phenotype in beta-thalassemia. Hematology. 2008;13(4):247–52. https://doi.org/10.1179/102453308X316031
  11. Cao A, Galanello R. Beta-thalassemia. Genet Med. 2010;12(2):61–76. https://doi.org/10.1097/GIM.0b013e3181cd68ed
  12. Cappellini N, Cohen A, Eleftheriou A, Piga A, Porter J. Guidelines for the clinical management of thalassemia. Thalassemia International Federation; 2000.
  13. Sripichai O, Munkongdee T, Kumkhaek C, Svasti S, Winichagoon P, Fucharoen S. Coinheritance of the different copy numbers of alpha-globin gene modifies severity of beta-thalassemia/Hb E disease. Ann Hematol. 2008;87(5):375–9. https://doi.org/10.1007/s00277-007-0407-2





Copyright (c) 2018 Pustika Amalia Wahidiyat, Sudigdo Sastroasmoro, Suthat Fucharoen, Iswari Setianingsih, Siti Ayu Putriasih

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

All articles and issues in Medical Journal of Indonesia have unique DOI number registered in Crossref.
 
Romeo
 
http://mji.ui.ac.id/journal/index.php/mji/pages/view/stat 
Unique Visitors