# Splenectomy in Thalassemia: The Role of Surgery as an Adjunct to Medical Management

## TL;DR

This paper examines the safety and effectiveness of splenectomy in beta thalassemia patients with hypersplenism, finding it to be a low-risk procedure with no mortality in the short term.

## Contribution

The study provides clinical evidence on the role of open splenectomy as a safe adjunct to medical management in beta thalassemia.

## Key findings

- All seven patients underwent splenectomy with minimal morbidity and no mortality.
- Three patients also had cholecystectomy due to gallstones.
- Short-term outcomes were positive, but long-term effects remain unassessed due to limited follow-up.

## Abstract

Introduction

Beta thalassemia is a hemoglobinopathy characterized by defective production of the beta chain of hemoglobin, leading to irreversible destruction of RBCs, splenomegaly, pancytopenia, and a requirement for multiple transfusions. This condition necessitates iron chelation therapy, and splenectomy is often performed to manage hypersplenism.

Methods

This report includes a series of seven diagnosed cases of beta thalassemia with hypersplenism, all of whom underwent open splenectomy. Preoperative transfusions were administered to achieve target hemoglobin and platelet counts of 9 g/dL and 50,000/µL, respectively.

Results

The study included seven patients diagnosed with beta thalassemia, all of whom underwent open splenectomy. Among these, three patients also had concomitant cholecystectomy due to the presence of gallstones. The primary indication for performing splenectomy was hypersplenism. Preoperative transfusions were administered to ensure target hemoglobin levels of 9 g/dL and platelet counts of 50,000/µL. All patients were successfully discharged with minimal morbidity and no reported mortality. The longest follow-up period observed in this series was 10 months post-splenectomy, which limited the assessment of long-term effects.

Conclusion

Open splenectomy for hypersplenism in patients with beta thalassemia appears to be a safe and effective procedure with minimal short-term morbidity and no mortality observed in this series. However, due to the limited follow-up duration, the long-term effects of splenectomy in these patients could not be evaluated. Further studies with longer follow-up are needed to assess the long-term outcomes of splenectomy in beta thalassemia patients.