Splenectomy is significantly associated with thrombosis but not with pulmonary hypertension in patients with transfusion-dependent thalassemia: a meta-analysis of observational studies.

Introduction: Thromboembolism (TE) and pulmonary hypertension (PH) constitute frequently occurring complications in patients with transfusion-dependent thalassemia and have been associated with splenectomy in different studies. Nevertheless, the size of the possible association varies greatly in literature. Herein, we sought to provide pooled effect estimates regarding the impact of splenectomy on TE and PH in transfusion dependent thalassemia (TDT) by retrieving relevant, available studies. Methods: We systematically searched articles published in PubMed, Cochrane library, Scopus and gray literature from inception until the 30th of May, 2023. Pooled estimates in terms of odds ratios (OR) and 95% confidence intervals (CI) were calculated according to outcome measures. Risk of bias and quality of studies were evaluated. Results: Regarding TE, 4 studies were selected for meta-analysis and the pooled data demonstrated that splenectomy was significantly associated with this outcome in TDT patients [OR = 4.08, 95% CI (1.03, 16.11), p = 0.04]. On the other hand, we pooled data from seven investigating PH, and, interestingly, the quantitative analysis revealed no association between splenectomy and PH [OR = 1.76, 95% CI (0.91, 3.41), p = 0.1]. Conclusion: Splenectomy is associated with higher risks of TE, but not with PH in patients with TDT.

Kidney transplantation in an adult with transfusion-dependent beta thalassemia: A challenging case report and literature review.

The markedly increased survival of transfusion-dependent beta thalassemia patients has led to the recognition of new complications, such as renal disorders. Kidney transplantation is nowadays the preferred treatment option for end-stage kidney disease (ESKD). We describe a case of a 49-year-old woman with ß-Transfusion Dependent Thalassemia, who developed ESKD as a result of focal segmental glomerulosclerosis and received a deceased-donor kidney transplant following hemodialysis for over a decade. The particular challenges of this case are discussed, including the long-term survival in hemodialysis. Our patient had to overcome multiple obstacles, including hypercoagulability issues presented in the form of thromboembolism, infections, such as hepatitis C and gastroenteritis, and the acute T-cell-mediated rejection, which had to be managed postoperatively. A review of the current literature revealed only one previous report of a thalassemia patient who successfully underwent renal transplantation. More than a year after the transplantation our patient presents with a normal glomerular filtration rate (GFR=62ml/min/1.73m2) and creatinine level (Cr=0.96mg/dL) and is transfused every 3 weeks. In conclusion, renal transplantation is possible in patients with TDT and should not be discouraged. Regular transfusions and optimal follow-up for the elimination of post-transplant complications are required.