Iron overload (IOL), resulting from regular or intermittent blood transfusions or from increasing dietary iron absorption can cause serious and life-threatening complications. Patients at risk of IOL include those with inherited anaemias such as transfusion-dependent thalassaemia (TDT) and non-transfusion-dependent thalassaemia (NTDT), transfused sickle cell disease (SCD) and rarer anaemias such as congenital sideroblastic anaemia (CSA), congenital dyserythropoietic anaemia (CDA), Diamond-Blackfan anaemia (DBA) as well as red cell enzymopathies, membrane disorders and defects in haem synthesis pathways. The United Kingdom has approximately 15 000 patients with these disorders and diagnosis and management of IOL is important in minimising morbidity and mortality. Other disorders that are associated with IOL such as hereditary haemochromatosis or acquired anaemias such as the myelodysplastic syndromes are not covered by this guideline.
The extent and severity of IOL is affected by both the underlying disorder and the intensity and duration of transfusion. Patients on regular top-up transfusions are at most risk whilst those on intermittent transfusions develop IOL more slowly. In the absence of blood transfusion, sickle cell disorders tend not to accumulate excess iron: however, manual and automated exchange transfusion may result in mild degrees of IOL or even iron deficiency.1-3
Declaration of Interests
The BSH paid the expenses incurred during the writing of this guidance. None of the authors had conflicts of interest to declare. All authors have made a declaration of interests to the BSH and Task Force Chairs which may be viewed on request.