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 complic...

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Bibliographic Details
Published in:British journal of surgery 2011-06, Vol.98 (6), p.751-760
Main Authors: Saad, G. S. Abi, Musallam, K. M., Taher, A. T.
Format: Article
Language:English
Subjects:
Anemia - prevention & control
beta-Thalassemia - complications
beta-Thalassemia - surgery
Blood Transfusion - methods
Chelation Therapy - methods
Cholecystectomy - methods
Facial Bones - surgery
Gallstones - surgery
Humans
Intraoperative Care - methods
Intraoperative Complications - prevention & control
Iron Overload - prevention & control
Leg Ulcer - surgery
Osteoporotic Fractures - surgery
Postoperative Complications - prevention & control
Splenectomy - adverse effects
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Summary: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. Copyright © 2011 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. All surgeons should know about this
ISSN:0007-1323
1365-2168
DOI:10.1002/bjs.7533