PREDISPOSING FACTORS TO INFECTIONS IN THALASSEMIA SYNDROME PATIENTS
Main Article Content
Keywords
Anemia and thalassemia syndromes, Iron overload, Infections, Iron overload; Thalassemia, Thalassaemia
Abstract
Introduction: Thalassemia is an inherited blood disorder characterized by defective hemoglobin production, leading to chronic anemia. Patients, particularly those who are transfusion-dependent, face a heightened risk of infections due to disease-related factors like anemia and treatment-related complications such as iron overload and splenectomy. This study explores the factors contributing to infections in thalassemia patients to improve management strategies.
Methods: A retrospective analysis was conducted on 303 patients with thalassemia at a tertiary care center from 2007 to 2022. Data were collected on demographics, transfusion dependency, splenectomy status, ferritin levels, vaccination history, and culture results. Logistic regression analysis was used to identify infection risk factors, with significance set at p < 0.05.
Results: Out of 303 patients, 72 (23.8%) experienced culture-positive infections, with Escherichia coli being the most isolated pathogen. Patients with infections had significantly higher ferritin levels and were less likely to be on chelation therapy. Combined chelation therapy, however, showed a protective effect (OR: 0.02, p = 0.01). Female patients were more susceptible to infections (OR: 2.94, p = 0.04), while mortality was notably higher in patients with positive cultures (22% vs. 3%, p < 0.001).
Conclusion: This study highlights the strong link between iron overload, chelation and risk of infection in thalassemia patients. Effective management, including proper chelation therapy and monitoring ferritin levels, is critical for reducing infections and improving outcomes. Further studies are needed to confirm these findings and guide future management strategies.
Downloads
Abstract 67
PDF Downloads 17
HTML Downloads 9
References
1. Khan I, Shaikh H. Beta Thalassemia Major (Cooley Anemia). StatPearls. Treasure Island (FL): StatPearls Publishing
Copyright © 2025, StatPearls Publishing LLC.; 2025.
2. Rao E, Kumar Chandraker S, Misha Singh M, Kumar R. Global distribution of β-thalassemia mutations: An update. Gene. 2024;896:148022.
3. Viprakasit V, Ekwattanakit S. Clinical Classification, Screening and Diagnosis for Thalassemia. Hematol Oncol Clin North Am. 2018;32(2):193-211.
4. Musallam KM, Cappellini MD, Coates TD, Kuo KHM, Al-Samkari H, Sheth S, et al. Αlpha-thalassemia: A practical overview. Blood Rev. 2024;64:101165.
5. Patterson S, Singleton A, Branscomb J, Nsonwu V, Spratling R. Transfusion Complications in Thalassemia: Patient Knowledge and Perspectives. Front Med (Lausanne). 2022;9:772886.
6. Lal A, Wong T, Keel S, Pagano M, Chung J, Kamdar A, et al. The transfusion management of beta thalassemia in the United States. Transfusion. 2021;61(10):3027-39.
7. Pinto VM, Forni GL. Management of Iron Overload in Beta-Thalassemia Patients: Clinical Practice Update Based on Case Series. Int J Mol Sci. 2020;21(22).
8. Cianciulli P. Iron chelation therapy in thalassemia syndromes. Mediterr J Hematol Infect Dis. 2009;1(1):e2009034.
9. Vichinsky E, Neumayr L, Trimble S, Giardina PJ, Cohen AR, Coates T, et al. Transfusion complications in thalassemia patients: a report from the Centers for Disease Control and Prevention (CME). Transfusion. 2014;54(4):972-81; quiz 1.
10. Atmakusuma TD, Girson R, Koesnoe S. Correlations between Iron Load and CD4 in Adult Transfusion-Dependent Beta Thalassemia. Anemia. 2021;2021(1):5549503.
11. Vento S, Cainelli F, Cesario F. Infections and thalassaemia. Lancet Infect Dis. 2006;6(4):226-33.
12. Sharma A, Easow Mathew M, Puri L. Splenectomy for people with thalassaemia major or intermedia. Cochrane Database Syst Rev. 2019;9(9):Cd010517.
13. Tahir F, Ahmed J, Malik F. Post-splenectomy Sepsis: A Review of the Literature. Cureus. 2020;12(2):e6898.
14. Luu S, Spelman D, Woolley IJ. Post-splenectomy sepsis: preventative strategies, challenges, and solutions. Infect Drug Resist. 2019;12:2839-51.
15. Alqahtani MM AA, Asirri SA, Alwuthaynani MT, Ishaq YMA, Hasan ER, Almasri WA, Alkhalil AM, Murdhimah AHA, Albalawai IN. Prevalence of thalassemia in Saudi Arabia: a systematic review and meta-analysis. IJMDC. 2024;8(10):2903-12.
16. Connor L, Dean J, McNett M, Tydings DM, Shrout A, Gorsuch PF, et al. Evidence-based practice improves patient outcomes and healthcare system return on investment: Findings from a scoping review. Worldviews Evid Based Nurs. 2023;20(1):6-15.
17. Alzhrani FS, Algethmi FA, Makin MA, Barayan NA, Hilal RM, Alnakhli SM. Prevalence and risk factors of severe bacterial infections in thalassemia patients. Current Pediatric Research. 2020;24:264-9.
18. Ahmed Kiani R, Anwar M, Waheed U, Asad MJ, Abbasi S, Abbas Zaheer H. Epidemiology of Transfusion Transmitted Infection among Patients with β-Thalassaemia Major in Pakistan. J Blood Transfus. 2016;2016:8135649.
19. Wang SC, Lin KH, Chern JP, Lu MY, Jou ST, Lin DT, et al. Severe bacterial infection in transfusion-dependent patients with thalassemia major. Clin Infect Dis. 2003;37(7):984-8.
20. Sakran W, Levin C, Kenes Y, Colodner R, Koren A. Clinical spectrum of serious bacterial infections among splenectomized patients with hemoglobinopathies in Israel: a 37-year follow-up study. Infection. 2012;40(1):35-9.
21. Teawtrakul N, Jetsrisuparb A, Sirijerachai C, Chansung K, Wanitpongpun C. Severe bacterial infections in patients with non-transfusion-dependent thalassemia: prevalence and clinical risk factors. Int J Infect Dis. 2015;39:53-6.
22. Scott CR, Holbein BE, Lehmann CD. Iron should be restricted in acute infection. Front Biosci (Landmark Ed). 2020;25(4):673-82.
23. Rahav G, Volach V, Shapiro M, Rund D, Rachmilewitz EA, Goldfarb A. Severe infections in thalassaemic patients: prevalence and predisposing factors. Br J Haematol. 2006;133(6):667-74.
24. Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Guidelines for the Clinical Management of Thalassaemia. Nicosia (CY): Thalassaemia International Federation
© 2008 Thalassaemia International Federation.; 2008.
25. Cherayil BJ. Iron and immunity: immunological consequences of iron deficiency and overload. Arch Immunol Ther Exp (Warsz). 2010;58(6):407-15.
26. Kao JK, Wang SC, Ho LW, Huang SW, Chang SH, Yang RC, et al. Chronic Iron Overload Results in Impaired Bacterial Killing of THP-1 Derived Macrophage through the Inhibition of Lysosomal Acidification. PLoS One. 2016;11(5):e0156713.
27. Neilands JB. Siderophores: structure and function of microbial iron transport compounds. J Biol Chem. 1995;270(45):26723-6.
28. Kontoghiorghes GJ, Efstathiou A, Kleanthous M, Michaelides Y, Kolnagou A. Risk/benefit assessment, advantages over other drugs and targeting methods in the use of deferiprone as a pharmaceutical antioxidant in iron loading and non iron loading conditions. Hemoglobin. 2009;33(5):386-97.
29. Belmont A, Kwiatkowski JL. Deferiprone for the treatment of transfusional iron overload in thalassemia. Expert Rev Hematol. 2017;10(6):493-503.
30. Karagün BŞ. ADVANCES IN THALASSEMIA MANAGEMENT AND CHELATION THERAPY. Hematology, Transfusion and Cell Therapy. 2024;46:S5-S6.
31. SMITH CH, ERLANDSON ME, STERN G, SCHULMAN I. The Role of Splenectomy in the Management of Thalassemia. Blood. 1960;15(2):197-211.
32. Wiedermann CJ. Hypoalbuminemia as Surrogate and Culprit of Infections. Int J Mol Sci. 2021;22(9).
33. Shaalan MG, Hassan MK, Al-Shanoof HJ, Al Naama LM. Renal Dysfunction in Pediatric Patients in Iraq With β-Thalassemia Major and Intermedia. Cureus. 2022;14(9):e29183.
34. Mahmoud AA, Elian DM, Abd El Hady NM, Abdallah HM, Abdelsattar S, Khalil FO, et al. Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major. Children (Basel). 2021;8(2).
35. Demosthenous C, Vlachaki E, Apostolou C, Eleftheriou P, Kotsiafti A, Vetsiou E, et al. Beta-thalassemia: renal complications and mechanisms: a narrative review. Hematology. 2019;24(1):426-38.
36. Srevatsa K, Kangle RP, Jali S. Role of Prothrombin time International normalized ratio and activated partial thromboplastin time in beta thalassemia major: A cross sectional study. Indian Journal of Pathology and Oncology. 2019.
37. Zurlo MG, De Stefano P, Borgna-Pignatti C, Di Palma A, Piga A, Melevendi C, et al. Survival and causes of death in thalassaemia major. Lancet. 1989;2(8653):27-30.
38. Borgna-Pignatti C, Ventola M, Friedman D, Cohen AR, Origa R, Galanello R, et al. Seasonal variation of pretransfusion hemoglobin levels in patients with thalassemia major. Blood. 2006;107(1):355-7.
39. Fucharoen S, Viprakasit V. Hb H disease: clinical course and disease modifiers. Hematology Am Soc Hematol Educ Program. 2009:26-34.
40. Songdej D FS. INFECTIONS AND HAEMOGLOBIN H DISEASE. Amid A LA, Coates TD, et al., editors., editor. Nicosia (Cyprus): Thalassaemia International Federation; 2023.
Article Sidebar
Article Details
How to Cite
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Transfer of Copyright and Permission to Reproduce Parts of Published Papers. Authors will grant copyright of their articles to the Institute of Hematology, Catholic University, Rome . No formal permission will be required to reproduce parts (tables or illustrations) of published papers, provided the source is quoted appropriately and reproduction has no commercial intent. Reproductions with commercial intent will require written permission and payment of royalties.