Malignancy

Compared with the general population, people with coeliac diseaseA condition where a person is unable to eat gluten as it makes their body attack itself. have an increased risk of developing the following cancers (1):

• adenocarcioma of the small bowel
• a very rare type of non Hodgkin’s lymphoma of the small bowel called enteropathy associated T cell lymphoma (EATL)
• other types of non Hodgkin’s lymphoma
• Hodgkin’s lymphoma.

However, overall the rates are low (1) and the majority of people with coeliac disease do not develop lymphoma or the other cancers that are associated with coeliac disease. 

It is recommended that serological testing should be offered to any children or adults with lymphoma (1).

What is lymphoma?

Lymphoma is a type of cancer that affects the lymphatic system. The lymphatic system is made up of the lymph glands, lymphatic vessels and the spleen. The lymphatic system helps the body fight infection and filters body fluid.

What is non Hodgkin’s lymphoma?

Non Hodgkin’s lymphoma can affect any part of the body including the small bowel, stomach and bones. Enteropathy associated T cell lymphoma is a particular type of non Hodgkin’s lymphoma.

What is enteropathy associated T cell lymphoma?

Enteropathy associated T cell lymphoma (EATL) is a very rare type of non Hodgkin’s lymphoma affecting around 1 in a million people in the general population (2). It makes up less than 1% of all non-Hodgkin’s lymphomas. The main symptoms linked with EATL are gut problems such as chronic diarrhoea, abdominal pain and unexplained weight loss. People with coeliac disease have a slightly higher risk of developing EATL because this affects the same part of the gut that is affected in coeliac disease. However, even with this slightly increased risk, overall the likelihood of developing this type of cancer is still very rare.

Risk of malignancy after starting the gluten-free diet

Following a strict glutenA protein that is found in the cereals wheat, barley and rye. -free diet is protective against malignancy and research suggests that the risk of malignancy decreases with time from diagnosis of coeliac disease to nearly the same as occurs in the general population.

One study showed that the increased risk was most highly related to the first year after diagnosis (3).

A study by Elfstrom and colleagues found that although coeliac disease, inflammation and latent coeliac disease all increase the risk of gastrointestinal malignancy in the first year after diagnosis, there is no increase in risk after this (4).

Dermatitis herpetiformis

Evidence suggesting that people with dermatitis herpetiformis also have an increased risk of cancer, particularly lymphoma, is conflicting with some studies showing that there is no increased risk (5).

Why is there a higher risk of small bowel cancers?

We do not know the cause of small bowel cancers. One of the mechanisms that could be involved is the increased turnover of various types of cells in the bowel wall, where there is active inflammation and the villiYour villi stick out like tiny fingers from the wall of your gut. If you have coeliac disease and are not following a gluten-free diet, these become flat and you have difficulty absorbing nutrients. of the gut lining are trying to regenerate. 

The increased risk of small intestinal cancer in coeliac disease could be due to persistent inflammation, perhaps in conjunction with impairment in the mucosal lining and tight junctions which may result in the production of new tumors (4).

What about other cancers?

There appears to be a lower risk of developing breast cancer and lung cancer in people with coeliac disease. Although further research is needed to understand the reasons for this, the lower association with lung cancer may be because people with coeliac disease are less likely to smoke (3).

Refractory coeliac disease

Those who have been diagnosed with refractory coeliac disease are at an increased risk of developing EATL.

If your patients are concerned or have any questions about cancer risk they can contact our HelplineOur Helpline is staffed by dietitians and food experts. You can call them on 0845 305 2060., speak to their GPGeneral Practitioner, or local doctor or contact one of the cancer charities such as Cancer Research UK and the Lymphoma Association.

References:

1. National Institute for Health and Clinical Excellence (NICENational Institute for Health and Clinical Excellence - an independent organisation responsible for providing national guidance on promoting good health and preventing and treating ill health. NICE had produced a clinical guideline on the recognition and assessment of coeliac disease.) guidelines (2009) Recognition and assessment of coeliac disease http://www.nice.org.uk/nicemedia/pdf/CG86FullGuideline.pdf
2. Verbeek WH, Van De Water JM, Al-Toma A et al (2008) Incidence of enteropathy associated T-cell lymphoma: a nation-wide study of a population based registry in The Netherlands. Scandinavian Journal of Gastroenterology 43: 1322-1328
3. West J, Logan RF, Smith CF et al (2004) Malignancy and mortality in people with coeliac disease: population based cohort study. BMJ 329 (7468): 716 – 719
4. Elfstrom P, Granath F, Ye W et al (2011) Low risk of gastrointestinal cancer among patients with coeliac disease, inflammation or latent coeliac disease. Clinical Gastroenterology and Hepatology doi: 10.1016/j.cgh.2011.06.029
5. Lewis NR, Logan RFA, Hubbard RB et al (2008) No increase in risk of fracture, malignancy or mortality in dermatitis herpetiformis: a cohort study. Alimentary Pharmacology and Therapeutics 27 (11): 1140 - 1147