Outcome following Splenectomy in young Thalassemic patients aged five to eighteen years – a single centre observational study

Year 2023, Volume: 16 Issue: 1, 6 - 10, 31.01.2023

Dibyashree Paul , Arkaprovo Roy Shuchismita Chakraborty Alamgir Hossain

Abstract

Excessive destruction of abnormal red blood cells and resultant extra-medullary haematopoiesis in be-ta thalassemia major leads to splenomegaly, hypersplenism and increased requirement of blood transfusion, which often necessitate splenectomy.
To evaluate the outcome of splenectomy among patients with beta thalassemia major
A retrospective cross-sectional study was conducted on 26 beta thalassemia major patients, in the age group of 5 to18 years, who underwent elective splenectomy in a tertiary hospital between April, 2016 and May, 2018. Their pre-operative, peri-operative and post-operative follow up data were collected from a prospectively maintained database and analysed.
Out of 26 patients, 8 (30.8%) were female and 18 (69.2%) male. Mean age of our study population was 9.3 years. Mean haemoglobin at the time of admission was 5.76 +/- 0.49 g/dl and mean pre-operative blood transfusion requirement was 31.77 +/- 9.34 units/yr. Median length of post-operative hospital stay was 6 days. Immediate post-operative complications included haemorrhage in 1 (3.8%) patient, sepsis in 1 (3.8%), pulmo-nary complication in 1 (3.8%), wound infection in 2 (7.7%) and death of 2 (7.7%) patients. 19 (73.1%) patients did not encounter any post-operative complication. The mean blood transfusion requirement after splenectomy, as assessed in median follow-up over 26 months, was 13.00+/-7.15 units/yr.
In conclusion, for patients with beta thalassemia major being managed in resource limited setup, splenectomy can be considered as an effective procedure for reducing the blood transfusion requirement and hence, prevent its associated adverse effects.

Keywords

Thalassemia, splenectomy, outcome

References

• 1. Ghai OP. Essential pediatrics. 8th ed. New Delhi: CBS; 2013, p:341.
• 2. Harrison. Principles of internal medicine. 19th ed. United States of America: McGraw-Hill; 2015.
• 3. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood. 2011;118:3479–3488.
• 4. Trompeter S, Cohen A. Blood transfusion. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V, editors. Guidelines for the man-agement of transfusion dependent thalassae-mia (TDT),3rd ed. Nicosia: Thalassaemia Inter-national Federation; 2014, p:28–41.
• 5. Bansal D, Totadri S. Common hematological disorders in children. Indian J Pediatr. 2014;81:42–50.
• 6. Taher A, Tyan PI. The spleen. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V, editors. Guidelines for the management of transfusion dependent thalassaemia (TDT), 3rd ed. Nicosia: Thalassaemia International Federa-tion; 2014, p:126–133.
• 7. Luu et al. Post-splenectomy sepsis: preventative strategies, challenges, and solutions. Infect Drug Resist. 2019;12:2839-2851.
• 8. Zarina AL, Norazlin KN, Hamidah A, Aziz DA, Zulkifli SZ, Jamal R. Spectrum of infections in splenectomised thalassaemia patients. Med J Malaysia. 2010;65:284–286.
• 9. Al-Salem AH, Nasserulla Z. Splenectomy for children with thalassemia. Int Surg. 2002; 87:269–273. 10. Pecorari L, Savelli A, Guna CD, Fracchia S, Borgna-Pignatti C. The Role of Splenectomy in Thalassemia Major. An Update. Acta Pediatrica Mediterranea. 2008;24:57-60.
• 11. Porecha MM, Udani D, Mehta V, Gami A. Sple-nectomy in management of thalassemia major - a boon for the little Angel. Internet J Surg. 2010;24:1.
• 12. Cohen A, Gayer R,Mizanin J. Long-term effect of splenectomy on transfusion requirements in thalassemia major. Am J Hematol. 1989;30:254–256.
• 13. Konstadoulakis_MM, Lagoudianakis_E, Anto-nakis_PT, Albanopoulos_K, Gomatos_I, Stamou KM, et al. Laparoscopic versus open splenectomy in patients with beta thalassemia major. J Laparoend Ad Surg Tech. 2006;16(1):5-8.
• 14. Borgna-Pignatti C, Carnelli V, Caruso V, et al. Thromboembolic events in beta thalassemia major: an Italian multicenter study. Acta Hae-matol. 1998;99:76–79.
• 15. Cullingford GL, Watkins DN, Watts AD, Mallon DF. Severe late postsplenectomy infection. Br J Surg. 1991;78(6):716–721.
• 16. Standage BA, Goss JC. Outcome and sepsis after splenectomy in adults. Am J Surg. 1982;143(5): 545–548.