Splenectomy in Transfusion Dependant Thalassemia - Insights from an Urban Tertiary Care Center Experience

Abstract Background: Transfusion dependent Thalassemias require frequent blood transfusions for their well-being. Damaged or defective red blood cells are normally eliminated in the spleen. In Thalassemia there is a large quantity of defective red blood cells leads to an enlarged hyper functioning spleen. Splenectomy may thus prolong red blood cell survival & ultimately result in the reduced need for blood transfusions. However, it is associated with considerable long-term complications, including sepsis, thrombosis, and pulmonary hypertension. This study was conducted to study the indications, the long-term complications, and causes of mortality among patients with TDT who have undergone splenectomy in a low- and middle-income country (LMIC). Methods: Data was analysed retrospectively from the hospital records of transfusion dependent Thalassemia patients who underwent splenectomy between July 2017 to January 2024, Data collected included demographic and clinical characteristics, haematological parameters, transfusion requirements before splenectomy and at the last follow-up after splenectomy, complications, and mortality causes were studied. Results: Total data from 15 patients who underwent spleectomy were analyzed. Among these individuals, 11 were diagnosed with Thalassemia Major, 3 with Thalassemia Intermedia, and 1 presented with Hb-E--Beta Thalassemia. The median age at splenectomy was 14years (range 6–22). All of them underwent open splenectomy. The yearly transfusion volume decreased from 258 ml/kg pre-splenectomy (range 180−370) to 145 (range 140 –250)) post-splenectomy at the last follow-up, p < 0.001. Two patients were completely transfusion-free at the last follow-up. Complications included pulmonary hypertension in 3 (20 %), thrombosis in 2(13.3 %), and overwhelming post-splenectomy infection in 1 (6.6 %). The iron-overload-related complications included cardiomyopathy in 6 (40%), endocrinopathy in 4 (26.6%) and chronic liver disease in 3 (20 %). Only one patient died within one year of overhelming sepsis due to post spleenectomy. Conclusion: In our cohort Splenectomy improved mean Hemoglobin, cytopenias with reduction in mean PRC requirement without significant increase in morbidity/mortality. However decision of splenectomy should be individualized, considering risk benefit ratio.