ASSESSMENT OF SPLENIC FUNCTION AMONG TRANSFUSION DEPENDENT THALASSEMIA PATIENTS

Main Article Content

Muhammad Hamza
Salman Zahir
Aneela Khan
Sarwat Jahan
Daniyal Ahmed
Khansa Khan

Keywords

Thalassemia, Hypersplenism, Transfusion-Dependent Thalassemia, Splenectomy

Abstract

Transfusion-dependent thalassemia is a chronic disease that affects many individuals and has a significant impact on the healthcare system and society. This study sought to explore the prevalence and factors contributing to hypersplenism in transfusion-dependent thalassemia patients in the Khyber Pakhtunkhwa province of Pakistan. A single-center-based retrospective study was conducted on 50 randomly selected patients over a period of three months From February 2019 to April 2019 and data was analyzed using established statistical techniques. The results showed that all (n=50, 100%) of the participants developed hypersplenism at an average age of 6.86±1.9 years and underwent a splenectomy at an average age of 15.1±4.6 years. The study found that the average number of annual transfusions received by the patients was 19.1±7.7, with no significant change in transfusion frequency after splenectomy

Abstract 254 | pdf Downloads 104

References

1. Esmaeilzadeh F, Azarkeivan A, Emamgholipour S, Akbari Sari A, Yaseri M, Ahmadi B, et al. Economic Burden of Thalassemia Major in Iran, 2015. J Res Health Sci 2016;16(3):111-5.
2. Borgna-Pignatti C, Gamberini MR. Complications of thalassemia major and their treatment. Expert Rev Hematol 2011;4(3):353-66.
3. Lv Y, Lau WY, Li Y, Deng J, Han X, Gong X, et al. Hypersplenism: History and current status. Exp Ther Med 2016;12(4):2377-82.
4. Piga A, Serra M, Longo F, Forni G, Quarta G, Cappellini MD, Galanello R. Changing patterns of splenectomy in transfusion‐dependent thalassemia patients. American journal of hematology. 2011 Sep;86(9):808-10.
4. Sari TT, Gatot D, Akib AA, Bardosono S, Hadinegoro SR, Harahap AR, Idjradinata PS. Immune response of thalassemia major patients in Indonesia with and without splenectomy. Acta Medica Indonesiana. 2016 May 16;46(3).
5. Merchant RH, Shah AR, Ahmad J, Karnik A, Rai N. Post splenectomy outcome in β-thalassemia. The Indian Journal of Pediatrics. 2015 Dec;82:1097-100.
6. Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. The Lancet. 2011 Jul 2;378(9785):86-97.
7. William BM, Thawani N, Sae-Tia S, Corazza GR. Hyposplenism: a comprehensive review. Part II: clinical manifestations, diagnosis, and management. Hematology. 2007 Apr 1;12(2):89-98.
8. Mebius RE, Kraal G. Structure and function of the spleen. Nature reviews immunology. 2005 Aug 1;5(8):606-16.
9. Kruetzmann S, Rosado MM, Weber H, Germing U, Tournilhac O, Peter HH, Berner R, Peters A, Boehm T, Plebani A, Quinti I. Human immunoglobulin M memory B cells controlling Streptococcus pneumoniae infections are generated in the spleen. The Journal of experimental medicine. 2003 Apr 7;197(7):939-45.
10. William BM, Thawani N, Sae-Tia S, Corazza GR. Hyposplenism: a comprehensive review. Part II: clinical manifestations, diagnosis, and management. Hematology. 2007 Apr 1;12(2):89-98.
11. Taher AT, Musallam KM, Karimi M, El‐Beshlawy A, Belhoul K, Daar S, Saned M, Cesaretti C, Cappellini MD. Splenectomy and thrombosis: the case of thalassemia intermedia. Journal of Thrombosis and Haemostasis. 2010 Oct 1;8(10):2152-8.
12. Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V, editors. Guidelines for the management of transfusion dependent thalassaemia (TDT). Nicosia, Cyprus: Thalassaemia International Federation; 2014.
13. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, Nawarawong W. Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia. Heart. 2006 Oct 1;92(10):1467-72.
14. Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. British journal of haematology. 2000 Nov;111(2):467-73.
15. Bazi A, Shahramian I, Yaghoobi H, Naderi M, Azizi H. The role of immune system in thalassemia major: a narrative review. Journal of Pediatrics Review. 2018 Jul 10;6(2):29-36.
16. Osataphan N, Dumnil S, Tantiworawit A, Punnachet T, Hantrakun N, Piriyakhuntorn P, Rattanathammethee T, Hantrakool S, Chai-Adisaksopha C, Rattarittamrong E, Norasetthada L. The long-term efficacy in blood transfusions, hematologic parameter changes, and complications after splenectomy in patients with transfusion-dependent thalassemia. Transfusion and Apheresis Science. 2022 Dec 9:103620.
17. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood, The Journal of the American Society of Hematology. 2011 Sep 29;118(13):3479-88.
18. Sharma A, Mathew ME, Puri L. Splenectomy for people with thalassaemia major or intermedia. Cochrane Database of Systematic Reviews. 2019(9).
19. Taher AT, Musallam KM, Nasreddine W, Hourani R, Inati A, Beydoun A. Asymptomatic brain magnetic resonance imaging abnormalities in splenectomized adults with thalassemia intermedia. Journal of Thrombosis and Haemostasis. 2009 Jan 1;8(1):54-9.
20. Zhou YL, Zhang XH, Liu TN, Wang L, Yin XL. Splenectomy improves anaemia but does not reduce iron burden in patients with haemoglobin H Constant Spring disease. Blood Transfusion. 2014 Oct;12(4):471.
21. Sirachainan N. Thalassemia and the hypercoagulable state. Thrombosis research. 2013 Dec 1;132(6):637-41

Most read articles by the same author(s)