Treatment of dental and orthodontic problems in thalassaemia

Review question

We reviewed the evidence about how best to treat dental and orthodontic problems in people with thalassaemia.


Red blood cells make an oxygen-carrying pigment known as haemoglobin. In thalassaemia the haemoglobin is not normal, due to defects (mutations) in two specific types of genes, leading to the classification of alpha (α) or beta (β) thalassaemias. About 5% of the world population are carriers of the mutation that causes the α-globin gene to only function partially or not at all, for the β-globin gene the carrier rate is about 1.5%, and millions more are being identified each year. Both forms of thalassaemia are mainly found in the belt of countries stretching from sub-Saharan Africa, through the Mediterranean region and the Middle East, to South and South-East Asia. Currently, due to people moving from country to country, these disorders are now also found in many other parts of the world.

When people inherit two copies of the mutated gene, the defective haemoglobin in the red blood cells does not release oxygen normally into the body, resulting in the symptoms of thalassaemia. These defective cells build up in the body's organs and bone marrow cells, causing both tissue damage and cell death. This leads to anaemia, which is a decrease in red blood cells. The lack of oxygen due to anaemia can stop organs from working normally, so, depending on severity of the condition, blood transfusions are often needed to correct the drop in red blood cells. The body tries to compensate for this anaemia naturally by increasing the number of formative red blood cells which causes expansion of the bone marrow spaces. In the skull, cheekbones and jawbones, this expanding bone marrow leads to abnormal bony swellings, which results in jaw deformity and teeth not being in their correct position (known as malocclusion). Difficulties with speech, eating and appearance result from severe changes to the face and jaw. These visible features can cause distress in people with thalassaemia and have a negative impact on their quality of life.

Since people with thalassaemia inevitably have to focus on dealing with the serious impact of anaemia on their general health to survive and on managing complications related to its long-term treatment, they may neglect other dental problems like tooth decay, gum disease, infections, etc. As a result ordinary dental infections can become more severe and require advanced treatment. Before starting any kind of dental treatment in people with thalassaemia, practitioners need to consider both the underlying condition and the effects of the resulting anaemia or its treatment. Dental treatment may be particularly risky in people with thalassaemia who have had their spleen removed, which can make them more prone to infections.

Given the significant effects of dental and orthodontic problems on the lives of people with thalassaemia, it is important to find the best way to treat them effectively. However, due to a lack of information in scientific literature, there are no guidelines to suggest the best treatment plan. Therefore, we aimed to search for any evidence for treating dental and orthodontic complications in people with thalassaemia and make it available to healthcare providers as well as people with this condition.

Search date

The evidence is current to: 22 July 2019.

Trial characteristics

We screened the titles of 35,202 references, but none of them were found to be suitable for inclusion in our review.

Key results

There need to be high-quality randomised controlled trials (trials where different treatments are compared and people are chosen for one or the other treatment by chance) investigating different treatment options for dental and orthodontic complications in people with thalassaemia.

Authors' conclusions: 

We were unable to draw any conclusions due to the lack of available data and trials. This review highlights the need for conducting and appropriate reporting, of high-quality randomised controlled trials investigating the effectiveness of various treatment modalities for dental and orthodontic complications in people with thalassaemia.

Read the full abstract...

Thalassaemia is a quantitative abnormality of haemoglobin caused by mutations in genes controlling production of alpha or beta globins. Abnormally unpaired globin chains cause haemolytic anaemia by causing membrane damage and cell death within organ systems and destruction of erythroid precursors in the bone marrow. The life-long management of the general health effects of thalassaemia in affected individuals is a highly challenging issue in and of itself; and failure to deal with dental and orthodontic complications in people with thalassaemia exacerbates the public health, financial and personal burden posed by the condition. There exists a lack of evidence-based guidelines for care-seekers and providers to best deal with such dental and orthodontic complications in thalassaemia, which this review seeks to address.


The main objective of this review was to assess different methods to treat dental and orthodontic complications in people with thalassaemia.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We searched the reference lists of relevant articles and reviews.

Date of last search: 01 August 2019.

We also searched nine online databases (PubMed, Google Scholar,, WHO International Clinical Trials Registry Platform, Literature in the Health Sciences in Latin America and the Caribbean database, African Index Medicus, Index Medicus for South East Asia Region, Index Medicus for the Eastern Mediterranean Region, Indexing of Indian Medical Journals). We searched the reference lists of relevant articles and reviews and contacted haematologists, experts in fields of dentistry, organizations, pharmaceutical companies and researchers working in this field.

Date of last search: 22 July 2019.

Selection criteria: 

We searched for published or unpublished randomised controlled trials for treatment of dental and orthodontic complications in individuals diagnosed with thalassaemia, irrespective of phenotype, severity, age, gender and ethnic origin.

Data collection and analysis: 

Two review authors independently screened 35,202 titles from search results. We identified four unique randomised controlled trials, of which one seemed potentially relevant. Based on closer inspection, the trial was found not to be eligible for inclusion.

Main results: 

We did not find any relevant trials for inclusion in the review.