The surgeon and the patient with β-thalassaemia intermedia.

BACKGROUND

Unlike patients with β-thalassaemia major, where lifelong transfusion and iron chelation therapy are necessary for survival, patients with β-thalassaemia intermedia (TI) generally have a milder course and anaemia. The underlying pathophysiology of the disease still allows several complications to manifest. Surgical management during the course of the disease is common but relevant data from the literature have never been reviewed constructively. This aim of this review was to highlight this clinical entity to the surgeon, and ensure optimal and timely intervention.

METHODS

The review was based on potentially relevant studies identified from an electronic search of MEDLINE and PubMed databases. There were no language or publication year restrictions. References in published articles were also reviewed.

RESULTS

Surgical intervention is often essential to ensure optimal control of the associated morbidity in TI. Several general considerations are necessary before surgical intervention with regard to anaemia, cardiovascular disease, thromboembolic events and the effects of iron overload. Splenectomy, cholecystectomy, leg ulcers, fractures and extramedullary pseudotumours are the most commonly encountered surgical problems related to TI.

CONCLUSION

Awareness of TI and its associated morbidity is important so that appropriate preoperative care can occur.