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The craniofacial manifestations of beta thalassaemia are widely reported, and occur as a result of inadequate haematopoiesis leading to marrow space expansion. Secondarily, extramedullary haematopoiesis can occur throughout the body, although this is rarely reported in the paranasal sinuses. We discuss the systemic and craniofacial manifestations of beta thalassaemia major, with a focus on the radiological findings, as illustrated in a case of a 55-year-old male patient who was referred to the dental hospital as a result of complications of thalassaemia. This case report focuses on the effects of thalassaemia that may be encountered in a dental setting and highlights the radiological findings that clinicians should be aware of.
CPD/Clinical Relevance: Dental and craniofacial manifestations, and their management, are discussed for patients with beta thalassaemia.
Article
Thalassaemia is a genetic disorder involving abnormal haemoglobin formation.1 The haemoglobin molecule consists of both alpha and beta chains. In thalassaemia, the alpha or beta chains in the haemoglobin are defective as a result of mutation in the alpha or beta globin genes, resulting in a reduced oxygen-carrying capacity of the blood. This leads to overproduction of red blood cells and abnormal haemoglobin in the body. Thalassaemia can be described as either alpha or beta thalassaemia depending on which chains of the haemoglobin molecule are affected.2
There are several subtypes of the disease, including the minor, intermediate and major forms, which all present with varying severity. Beta thalassaemia is mainly found in Mediterranean, African and Asian populations.
Thalassaemias are a quantitative problem of haemoglobin synthesis where not enough ‘normal’ haemoglobin is produced to cope with the oxygenation demands of the body (ineffective erythropoiesis). In beta thalassaemias, there is also haemolysis of the defective circulating red blood cells. These phenomena together result in severe anaemia. The body may compensate for reduced oxygen carrying capacity through hyperplasia of bone marrow. Individuals with thalassaemia major often require regular blood transfusions to counteract the chronic anaemia that accompanies the disease.2
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