Type 1 Diabetes in Transfusion-Dependent Beta-Thalassemia Major a case series

Bikash Chandra  Nanda; Dr. Chakradhar  Majhi; Dr. Tuhin  Hati

doi:10.51168/sjhrafrica.v5i12.1483

Authors

Bikash Chandra Nanda Assistant Professor, Department of General Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur
Dr. Chakradhar Majhi 2. Professor & HOD, Department of General Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur
Dr. Tuhin Hati 3. PG Resident, Department of General Medicine, Veer Surendra Sai Institute of Medical Sciences and Research, Burla, Sambalpur

DOI:

https://doi.org/10.51168/sjhrafrica.v5i12.1483

Keywords:

Beta-Thalassemia, Type 1 Diabetes Mellitus, Iron Overload, Insulin Therapy

Abstract

Background: Regular blood transfusions, essential for managing transfusion-dependent beta-thalassemia major (TDT), frequently lead to iron overload, which can harm multiple organs, including the pancreas. Iron deposition in pancreatic beta-cells may result in Type 1 Diabetes Mellitus (T1DM). This case series examines the clinical characteristics, diagnostic findings, and management strategies for ten adult TDT patients who developed T1DM due to iron overload.

Methods: This study included 10 adult patients, aged between 18 and 35 years, presenting with TDT. Data collected encompassed fasting plasma glucose (FPG), HbA1c, serum ferritin levels, and chelation therapy adherence. All patients required chronic transfusion therapy and exhibited varying levels of compliance with iron chelation protocols.

Results: In this case series, ten patients with transfusion-dependent beta-thalassemia major (TDT) exhibited ferritin levels ranging from 2,600 to over 4,000 ng/mL and HbA1c levels between 6.5% and 9.2%, reflecting varying glycemic control. All patients had elevated fasting plasma glucose (FPG) levels, ranging from 110 to 200 mg/dL. Chelation therapy compliance was generally poor or suboptimal, influencing clinical outcomes. While most patients showed improvement or stabilization after treatment, one patient had limited improvement due to advanced disease complications.

Conclusion: Proactive monitoring for glucose abnormalities and rigorous adherence to iron chelation are essential to mitigate diabetes onset in TDT patients. Regular oral glucose tolerance tests (OGTT) and comprehensive care are recommended to address this high-risk population effectively.

References

Galanello, R., & Origa, R. (2010). Beta-thalassemia. Orphanet journal of rare diseases, 5, 1-15.

Kattamis, A., Kwiatkowski, J. L., & Aydinok, Y. (2022). Thalassaemia. The lancet, 399(10343), 2310-2324.

White, J. M., Byrne, M. A. R. G. A. R. E. T., Richards, R., Buchanan, T., Katsoulis, E. L. I. Z. A. B. E. T. H., & Weerasingh, K. A. L. I. Y. A. N. I. (1986). Red cell genetic abnormalities in Peninsular Arabs: sickle haemoglobin, G6PD deficiency, and alpha and beta thalassaemia. Journal of medical genetics, 23(3), 245-251.

White, J. M., Byrne, M., Richards, R., Buchanan, T., Sheik‐Yousouf, I., White, Y. S., & Frost, B. (1985). Thalassaemia genes in peninsular Arabs. British journal of haematology, 60(2), 269-278.

Baysal, E. (2011). Molecular basis of β-thalassemia in the United Arab Emirates. Hemoglobin, 35(5-6), 581-588.

Srinoun, K., Svasti, S., Chumworathayee, W., Vadolas, J., Vattanaviboon, P., Fucharoen, S., & Winichagoon, P. (2009). Imbalanced globin chain synthesis determines erythroid cell pathology in thalassemic mice. Haematologica, 94(9), 1211.

Taher, A. T., & Saliba, A. N. (2017). Iron overload in thalassemia: different organs at different rates. Hematology 2014, the American Society of Hematology Education Program Book, 2017(1), 265-271.

Azami, M., Sharifi, A., Norozi, S., Mansouri, A., & Sayehmiri, K. (2017). Prevalence of diabetes, impaired fasting glucose and impaired glucose tolerance in patients with thalassemia major in Iran: A meta-analysis study. Caspian journal of internal medicine, 8(1), 1.

Olivatto, G. M., Teixeira, C. R. D. S., Sisdelli, M. G., Zanetti, M. L., Silveira, R. C. D. C. P., & Gonçalves, C. V. (2019). Characterization of thalassemia major and diabetes mellitus patients at a reference center in Brazil. Hematology, Transfusion and Cell Therapy, 41, 139-144.

Töret, E., Karadaş, N. Ö., Gökçe, N. Ö., Kaygusuz, A., Karapınar, T. H., Oymak, Y., & Gözmen, S. (2018). Quality of life and depression in Turkish patients with β-thalassemia major: A cross-sectional study. Hemoglobin, 42(5-6), 326-329.

Voskaridou, E., & Terpos, E. (2004). New insights into the pathophysiology and management of osteoporosis in patients with beta thalassaemia. British Journal of Haematology, 127(2), 127-139.

Belhoul, K. M., Bakir, M. L., Kadhim, A. M., Dewedar, H. E., Eldin, M. S., & AlKhaja, F. A. (2013). Prevalence of iron overload complications among patients with β-thalassemia major treated at Dubai Thalassemia Centre. Annals of Saudi medicine, 33(1), 18-21.

De Sanctis, V., Soliman, A. T., Tzoulis, P., Daar, S., Fiscina, B., & Kattamis, C. (2021). Pancreatic changes affecting glucose homeostasis in transfusion dependent β-thalassemia (TDT): A short review. Acta Bio Medica: Atenei Parmensis, 92(3).

E Khamseh, M., Malek, M., Hashemi-madani, N., Ghassemi, F., Rahimian, N., Ziaee, A., ... & Faranoush, M. (2023). Guideline for the diagnosis and treatment of diabetes mellitus in patients with transfusion-dependent thalassemia. Iranian Journal of Blood and Cancer, 15(4), 293-303.

Blesia, V., Patel, V. B., Al-Obaidi, H., Renshaw, D., & Zariwala, M. G. (2021). Excessive iron induces oxidative stress promoting cellular perturbations and insulin secretory dysfunction in MIN6 beta cells. Cells, 10(5), 1141.

Serbis, A., Giapros, V., Tsamis, K., Balomenou, F., Galli-Tsinopoulou, A., & Siomou, E. (2023). Beta Cell Dysfunction in Youth- and Adult-Onset Type 2 Diabetes: An Extensive Narrative Review with a Special Focus on the Role of Nutrients. Nutrients, 15(9), 2217. https://doi.org/10.3390/nu15092217

Popoviciu, M. S., Kaka, N., Sethi, Y., Patel, N., Chopra, H., & Cavalu, S. (2023). Type 1 Diabetes Mellitus and Autoimmune Diseases: A Critical Review of the Association and the Application of Personalized Medicine. Journal of personalized medicine, 13(3), 422. https://doi.org/10.3390/jpm13030422

Harrison, A. V., Lorenzo, F. R., & McClain, D. A. (2023). Iron and the Pathophysiology of Diabetes. Annual review of physiology, 85, 339–362. https://doi.org/10.1146/annurev-physiol-022522-102832

Dmochowski K, Finegood DT, Francombe W, Tyler B, Zinman B. 1993. Factors determining glucose tolerance in patients with thalassemia major. J. Clin. Endocrinol. Metab 77:478–83

Tzoulis, P., Yavropoulou, M. P., Banchev, A., Modeva, I., Daar, S., & De Sanctis, V. (2023). Recent advancements in glucose dysregulation and pharmacological management of osteoporosis in transfusion-dependent thalassemia (TDT): an update of ICET-A (International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine). Acta bio-medica : Atenei Parmensis, 94(3), e2023178. https://doi.org/10.23750/abm.v94i3.14805