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

  • Pustika A. Wahidiyat Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
  • Sudigdo Sastroasmoro Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
  • Suthat Fucharoen Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Bangkok
  • Iswari Setianingsih Eijkman Institute for Molecular Biology, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
  • Siti A. Putriasih Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
Keywords: β-thalassemia/HbE, clinical criteria, Indonesia, scoring, Thailand
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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.

Author Biographies

Pustika A. Wahidiyat, Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
Head of Pediatric Hematology-Oncology Division, Department of Child Health, Faculty of Medicine, Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Sudigdo Sastroasmoro, Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
Department of Child Health, Faculty of Medicine, Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Suthat Fucharoen, Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Bangkok

Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Bangkok, Thailand

Iswari Setianingsih, Eijkman Institute for Molecular Biology, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
Eijkman Institute for Molecular Biology, Faculty of Medicine, Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Siti A. Putriasih, Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta
Department of Child Health, Faculty of Medicine, Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia

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

Published
2018-05-08
How to Cite
1.
Wahidiyat PA, Sastroasmoro S, Fucharoen S, Setianingsih I, Putriasih SA. Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia. Med J Indones [Internet]. 2018May8 [cited 2019Dec.9];27(1):26-2. Available from: http://mji.ui.ac.id/journal/index.php/mji/article/view/1779
Section
Clinical Research