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Thalassaemia - part 1: a clinical update for the dental team

Abstract

Thalassaemia is a genetic disorder that affects haemoglobin function. It is an abnormality of haemoglobin caused by mutation of genes related to alpha or beta globin chains that can be further subdivided into categories. These haematological conditions can vary from mild forms, which present as mild anaemia, to severe forms, that can become life-threatening. The window for dental treatment is often limited by several factors involving medical management, such as the need for antibiotic cover or blood transfusions. The lifetime management of the medical conditions are onerous and can place significant physical and psychological burden on the patient. This paper is part of a two-part series on thalassaemia. Part one focuses on the clinical manifestations patients may present with, treatment regimens and dental implications of such presentations. Part two explores the perspectives of thalassaemia patients on their dental experience.

Key points

  • Screening in patients with thalassaemia can enable detection of dental disease and facilitate early access to dental care services.

  • Thalassaemia patients can present with a range of orofacial manifestations, which may benefit from early dental intervention.

  • Evaluates emerging therapies for thalassaemia and identifies the dental implications this may trigger.

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References

  1. Boardman F K, Hale R. "I didn't take it too seriously because I'd just never heard of it": Experiential knowledge and genetic screening for thalassaemia in the UK. J Genet Couns 2019; 28: 141-154.

  2. Mulimani P, Abas A B, Karanth L, Colombatti R, Kulkarni P. Treatment of dental and orthodontic complications in thalassaemia. Cochrane Database Syst Rev 2019; DOI: 10.1002/14651858.CD012969.pub2.

  3. Boardman F K, Clark C, Jungkurth E, Young P J. Social and cultural influences on genetic screening programme acceptability: A mixed-methods study of the views of adults, carriers, and family members living with thalassemia in the UK. J Genet Couns 2020; 29: 1026-1040.

  4. Harteveld C L, Higgs D R. Alpha-thalassaemia. Orphanet J Rare Dis 2010; 5: 13.

  5. NHS. Thalassaemia carriers. 2019. Available at https://www.nhs.uk/conditions/thalassaemia/carriers/ (accessed November 2022).

  6. Hossain M S, Raheem E, Sultana T A et al. Thalassemias in South Asia: clinical lessons learnt from Bangladesh. Orphanet J Rare Dis 2017; 12: 93.

  7. Kattamis A, Forni G L, Aydinok Y, Viprakasit V. Changing patterns in the epidemiology of β-thalassemia. Eur J Haematol 2020; 105: 692-703.

  8. Viprakasit V, Ekwattanakit S. Clinical Classification, Screening and Diagnosis for Thalassemia. Haematol Oncol Clin North Am 2018; 32: 193-211.

  9. Muncie H L, Campbell J. Alpha and beta thalassemia. Am Fam Physician 2009; 80: 339-344.

  10. United Kingdom Thalassaemia Society. Standards for the Clinical Care of Children and Adults with Thalassaemia in the UK: Monitoring and Management of Iron Load. 2016. Available at https://www.stgeorges.nhs.uk/wp-content/uploads/2020/02/UKTS-adults-and-children-with-thalassaemia-guidelines-2016.pdf (accessed November 2022).

  11. Cutando Soriano A, Gil Montoya J A, de López-González Garrido J. Thalassemias and their dental implications. Med Oral 2002; 7: 36-45.

  12. De Sanctis V, Soliman A T, Elsedfy H et al. Osteoporosis in thalassemia major: an update and the I-CET 2013 recommendations for surveillance and treatment. Paediatr Endocrinol Rev 2013; 11: 167-180.

  13. Terpos E, Voskaridou E. Treatment options for thalassemia patients with osteoporosis. Ann N Y Acad Sci 2010; 1202: 237-243.

  14. Metheny L, de Lima M. Haematopoietic stem cell transplant with HLA-mismatched grafts: impact of donor, source, conditioning, and graft versus host disease prophylaxis. Expert Rev Haematol 2019; 12: 47-60.

  15. United Kingdom Thalassaemia Society. The importance of dental care. 2020. Available at https://ukts.org/wp-content/uploads/2021/02/dentalpullout.pdf (accessed July 2021).

  16. Sharma A, Easow Mathew M, Puri L. Splenectomy for people with thalassaemia major or intermedia. Cochrane Database Syst Rev 2019; DOI: 10.1002/14651858.CD010517.pub3.

  17. NHS. Sickle Cell Disease and Thalassaemia: Outpatient Clinic Review and Monitoring. 2019. Available at https://www.stgeorges.nhs.uk/wp-content/uploads/2020/02/SGH_HN502a_guidelines-Outpatient-monitoring-SCD-and-thalassaemia.pdf (acccessed May 2021).

  18. Mowla A, Karimi M, Afrasiabi A, De Sanctis V. Prevalence of diabetes mellitus and impaired glucose tolerance in beta-thalassemia patients with and without hepatitis C virus infection. Paediatr Endocrinol Rev 2004; 2: 282-284.

  19. United Kingdom Thalassaemia Society. Standards for the Clinical Care of Children and Adults with Thalassaemia in the UK: Management of Impaired Glucose Tolerance and Diabetes Mellitus. 2016. Available at https://www.stgeorges.nhs.uk/wp-content/uploads/2020/02/UKTS-adults-and-children-with-thalassaemia-guidelines-2016.pdf (accessed November 2022).

  20. Tzoulis P, Shah F, Jones R, Prescott E, Barnard M. Joint diabetes thalassaemia clinic: an effective new model of care. Hemoglobin 2014; 38: 104-110.

  21. Gunson H H, Rawlinson V I. HIV antibody screening of blood donations in the United Kingdom. Vox Sang 1988; 54: 34-38.

  22. Jirarattanasopa V, Hooncharoen P, Mekaewkunchorn A, Torcharus K. Effect of different transfusion regimens on craniofacial appearance and dentition in severe thalassemic children. Southeast Asian J Trop Med Public Health 2009; 40: 1371-1376.

  23. Sakka S D, Cheung M S. Management of primary and secondary osteoporosis in children. Ther Adv Musculoskelet Dis 2020; DOI: 10.1177/1759720X20969262.

  24. Scottish Dental Clinical Effectiveness Programme. Oral Health Management of Patients at Risk of Medication-related Osteonecrosis of the Jaw. 2017. Available at https://www.sdcep.org.uk/media/tsklutfj/sdcep-oral-health-management-of-patients-at-risk-of-mronj-guidance-in-brief.pdf (accessed May 2021).

  25. Helmi N, Bashir M, Shireen A, Ahmed I M. Thalassemia review: features, dental considerations and management. Electron Physician 2017; 9: 4003-4008.

  26. Master Anaesthesia. Thalassemia. Available at https://masteranesthesia.com/thalassemia/ (accessed November 2022).

  27. Maheri A, Sadeghi R, Shojaeizadeh D, Tol A, Yaseri M, Ebrahimi M. Associations between a health-promoting lifestyle and quality of life among adults with beta-thalassemia major. Epidemiol Health 2016; DOI: 10.4178/epih.e2016050.

  28. Cruz-Pamplona M, Margaiz-M Muñoz, Sarrión-Pérez M G. Dental considerations in patients with liver disease. J Clin Exp Dent 2011; 3: 127-134.

  29. Akcalı A, Yıldız M S, Akcalı Z, Huck O, Friedmann A. Periodontal condition of patients with Thalassemia Major: A systematic review and meta-analysis. Arch Oral Biol 2019; 102: 113-121.

  30. Chu C-H, Lee A H-C, Zheng L, Mei M L, Chan G C-F. Arresting rampant dental caries with silver diamine fluoride in a young teenager suffering from chronic oral graft versus host disease post-bone marrow transplantation: a case report. BMC Res Notes 2014; 7: 3.

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Acknowledgements

Medical illustrations are courtesy of Barts Health Trust.

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Both authors have been actively involved in the design of publication. Brasanyaa Raveendran was responsible for research and information analysis with the guidance of Mohammed Dungarwalla as the proofreader. The final publication has been reviewed and finalised by both authors.

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Correspondence to Brasanyaa Raveendran.

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Raveendran, B., Dungarwalla, M. Thalassaemia - part 1: a clinical update for the dental team. Br Dent J 233, 931–937 (2022). https://doi.org/10.1038/s41415-022-5302-7

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