Pancreatic Assessment in Beta Thalassemia Syndrome Patients of KPK

Muhammad Tariq Hamayun  Khan; Zubeda  Irshad; Muhammad Tariq Masood  Khan

doi:10.36283/ziun-pjmd14-4/012

Authors

Muhammad Tariq Hamayun Khan Burns & Plastic Surgery Center Hayatabad ,Peshawar, Pakistan. https://orcid.org/0000-0002-7116-5940
Zubeda Irshad Burns and Plastic Surgery Center. Hayatabad Medical College, Peshawar, Pakistan. https://orcid.org/0009-0003-2928-0683
Muhammad Tariq Masood Khan Pak International Medical College Hayatabad ,Peshawar, Pakistan. https://orcid.org/0000-0001-7841-7058

DOI:

https://doi.org/10.36283/ziun-pjmd14-4/012

Keywords:

Beta thalassemia, pancreas, iron overload, diabetes

Abstract

Background: Beta thalassemia is a hereditary hemoglobinopathy marked by ineffective erythropoiesis and progressive iron overload from early life. Iron deposition in the pancreas can impair both endocrine and exocrine function, increasing the risk of diabetes mellitus and pancreatic dysfunction. Regular pancreatic evaluation is essential to improve patient outcomes. This study assessed pancreatic function in beta thalassemia patients of Khyber Pakhtunkhwa (KP), Pakistan, and explored its association with transfusion frequency and iron overload.

Methods: This cross-sectional study was conducted in the Department of Hematology, Burns & Plastic Surgery Center, Hayatabad Peshawar, from January to December 2020. A total of 200 patients with beta thalassemia were evaluated. Clinical data included age, transfusion frequency, and serum ferritin levels. Laboratory investigations comprised fasting blood glucose, HbA1c, and pancreatic enzymes (amylase and lipase). Data were analyzed using SPSS, with significance set at p < 0.05.

Results: The mean age of patients was 16.5 ± 4.2 years. Ferritin levels >2500 ng/mL were significantly associated with impaired glucose tolerance and diabetes mellitus (p = 0.003). About 40% of patients had reduced amylase and lipase levels, suggesting exocrine pancreatic dysfunction. Patients receiving more than 12 transfusions per year demonstrated a higher prevalence of pancreatic dysfunction (p = 0.021). The findings indicate a direct effect of iron overload on pancreatic health.

Conclusion: Pancreatic dysfunction is common among beta thalassemia patients in KP due to iron accumulation. Routine screening for endocrine and exocrine insufficiency and optimized chelation therapy are essential to prevent long-term complications.

Author Biographies

Muhammad Tariq Hamayun Khan, Burns & Plastic Surgery Center Hayatabad ,Peshawar, Pakistan.

Department of Hematology and Pathology and Assistant Professor,
Zubeda Irshad, Burns and Plastic Surgery Center. Hayatabad Medical College, Peshawar, Pakistan.

Department of Microbiology and Pathology and Assistant Professor,
Muhammad Tariq Masood Khan, Pak International Medical College Hayatabad ,Peshawar, Pakistan.

Department of Hematology and Associate Professor,

References

1. Asmarian N, Kamalipour A, Hosseini-Bensenjan M, Karimi M, Haghpanah S. Prediction of heart and liver iron overload in β-thalassemia major patients using machine learning methods. Hemoglobin. 2022;46(6):303-307. doi:10.1080/03630269.2022.2158100

2. Meloni A, Saba L, Cademartiri F, Positano V, Pistoia L, Cau R. Cardiovascular magnetic resonance in β-thalassemia major: beyond T2. Radiol Med. 2024;129(12):1812-1822. doi:10.1007/s11547-024-01916-6

3. Ramanan V, Bhagyawant KR, Auti O, Gawande P. Assessment of cardiac, hepatic and pancreatic iron overload in transfusion-dependent thalassemia patients using T2* magnetic resonance imaging. Indian J Hematol Blood Transfus. 2024 Sep 17. doi:10.1007/s12288-024-01716-2

4. Mousa SO, Abd Alsamia EM, Moness HM, Mohamed OG. The effect of zinc deficiency and iron overload on endocrine and exocrine pancreatic function in children with transfusion-dependent thalassemia: a cross-sectional study. BMC Pediatr. 2021;21(1):1-9. doi:10.1186/s12887-021-02940-5

5. Musallam KM, Barella S, Origa R, Ferrero G, et al. Revisiting iron overload status and change thresholds as predictors of mortality in transfusion-dependent β-thalassemia: a 10-year cohort study. Ann Hematol. 2024;103(5):1234-1245. doi:10.1007/s00277-024-05000-1TIF+1TIF+1

6. Schwenzer NF, Machann J, Haap MM, Martirosian P, Schraml C, Liebig G, et al. T2* relaxometry in liver, pancreas, and spleen in a healthy cohort of one hundred twenty-nine subjects–correlation with age, gender, and serum ferritin. Invest Radiol. 2008;43(12):854-860. doi:10.1097/RLI.0b013e318185e9c5

7. Saggar K, Sobti P. MRI assessment of iron overload in thalassemia: an overview. Riv Ital Med Adolesc. 2013;11(1):1-5.

8. Maggio A, Capra M, Vitabile S, Rigano P, Cassarà F, Midiri M. Procedures for the evaluation of body iron burden in thalassemia major. Methods Mol Biol. 2010;610:21-32. doi:10.1007/978-1-60327-029-8_2

9. Tziomalos K, Perifanis V. Liver iron content determination by magnetic resonance imaging. World J Gastroenterol. 2010;16(13):1587-1597. doi:10.3748/wjg.v16.i13.1587

10. Parale GP, Pawar SS, Tapare VS. Assessment of LV diastolic function in patients with β-thalassemia major with special reference to E/Eann ratio. J Pediatr Hematol Oncol. 2009;31(1):69-73. doi:10.1097/MPH.0b013e31818e0c2c

11. Kushner JP, Porter JP, Olivieri NF. Secondary iron overload. Hematology Am Soc Hematol Educ Program. 2001;2001(1):47-61. doi:10.1182/asheducation-2001.1.47Agios

12. Tweed MJ. Pancreas, thyroid, parathyroid. In: Exposing the Hidden Dangers of Iron: What Every Medical Professional Should Know about the Impact of Iron on the Disease Process. 2004:67.

13. Sohail H, Fazal A, Lone KP, Kamran R, Ijaz F, Javed S, et al. Evaluation of bone density and leptin in thalassemic children. Biomedica. 2019;35(2):80-85.

14. De Sanctis V, Soliman AT, Daar S, Alansary N, Kattamis A, Skafida M, et al. A concise review on the frequency, major risk factors and surveillance of hepatocellular carcinoma in β-thalassemias: past, present and future perspectives and the ICET-A experience. Mediterr J Hematol Infect Dis. 2020;12(1):e2020006. doi:10.4084/MJHID.2020.006

15. Drakonaki EE, Maris TG, Maragaki S, Klironomos V, Papadakis A, Karantanas AH. Deferoxamine versus combined therapy for chelating liver, spleen and bone marrow iron in β-thalassemic patients: a quantitative magnetic resonance imaging study. Hemoglobin. 2010;34(1):95-106. doi:10.3109/03630260903580320

16. Marsella M, Borgna-Pignatti C. Transfusional iron overload and iron chelation therapy in thalassemia major and sickle cell disease. Hematol Oncol Clin North Am. 2014;28(4):703-727. doi:10.1016/j.hoc.2014.04.005

17. Lee TA, von Riedemann S, Tricta F. Cost-utility of chelators in transfusion-dependent β-thalassemia major patients: a review of the pharmacoeconomic literature. Expert Rev Pharmacoecon Outcomes Res. 2014;14(5):651-660. doi:10.1586/14737167.2014.950229

18. Farmakis D, et al. A short guide for the management of TDT: Thalassaemia International Federation; 2023. Available from: https://thalassaemia.org.cy/publications/tif-publications/a-short-guide-for-the-management-of-transfusion-dependent-thalassaemia-2022/TIF+2Agios+2TIF+2

19. Taher A, et al. Guidelines for the management of non-transfusion-dependent thalassaemias: updated version. Thalassaemia International Federation; 2023. Available from: https://thalassaemia.org.cy/publications/tif-publications/guidelines-for-the-management-of-non-transfusion-dependent-thalassaemias-updated-version/Agios

20. Musallam KM, Cappellini MD, Taher AT. Iron overload in non-transfusion-dependent thalassemia: a clinical perspective. Blood Rev. 2012;26(Suppl 1):S16-S19. doi:10.1016/S0268-960X(12)70006-5