Non-Hodgkin lymphoma is the sixth most common cancer type affecting adults in the UK. Photo / 123RF
Non-Hodgkin lymphoma is the sixth most common cancer type affecting adults in the UK, with around 14,200 people diagnosed with the disease every year, according to the charity Lymphoma Action. It is less well-known than other cancers.
This week, Jurassic Park actor Sam Neill was in the news, discussing his treatment for angioimmunoblastic T-cell lymphoma, a rare and aggressive form of the non-Hodgkin lymphoma subtype of blood cancers.
“Lymphoma sometimes flies under the radar,” says Dallas Pounds, director of services at Lymphoma Action. “One of the things we do is try and raise awareness of the different signs and symptoms so that people go and seek medical help earlier.”
Here are some facts and figures about the disease.
In short, non-Hodgkin lymphoma is a cancer of the lymphatic system, the intricate network of thin tubes and glands that transport a fluid called lymph around the body. In healthy people, lymph is vital because it carries white blood cells called lymphocytes to the site of infections, enabling the immune system to keep pathogens under control.
In patients with non-Hodgkin lymphoma, some of these lymphocytes begin to divide prematurely, affecting their ability to fight off infections. Instead, the abnormal lymphocytes collect in places like the lymph nodes or bone marrow where they can develop into tumours. Because lymph is so ubiquitous in the body, non-Hodgkin lymphoma can begin in almost any organ, with tumours often commonly found in the neck, liver or spleen.
Overall, there are more than 60 different subtypes of non-Hodgkin lymphoma. According to Dr Dima El-Sharkawi, a consultant haematologist at the Royal Marsden NHS Foundation Trust, the disease can be broadly categorised into high-grade or low-grade.
“This refers to how quickly the cells are dividing essentially,” she says. “For high grade, the cells are dividing more quickly, and the patients typically present as being more unwell. However, chemotherapies are also more effective on high-grade lymphomas and are potentially curative because chemo works by killing rapidly dividing cells.”
El-Sharkawi says that while low-grade forms of non-Hodgkin lymphoma tend to progress more slowly, they are typically not possible to eliminate completely.
“We’ve got plenty of treatments that can control and reduce the disease, but they don’t get rid of it completely,” she says.
Who is at risk of non-Hodgkin lymphoma?
While these cancers are more common with age, with 35 per cent of people diagnosed with non-Hodgkin lymphoma being 75 or older, a lot of the time doctors are unsure of the precise cause.
“One of the common questions that patients will ask me is ‘Why do I have this?’ and I genuinely have no answer for them,” says Prof Graham Collins, advanced therapy lead at private healthcare provider GenesisCare and national lymphoma expert. “There’s been lots of research done looking at causes of non-Hodgkin lymphoma, and there are a few, very specific subtypes where you can identify the cause, but for the vast majority of patients, we have no idea.”
Because lymphomas are cancers relating to a malfunctioning immune system, people who are immunosuppressed, such as transplant patients, or those with an autoimmune disease, such as rheumatoid arthritis or lupus are more vulnerable.
In the US, lawsuits have attempted to prove a link between environmental pesticides such as the weedkiller Roundup and the development of non-Hodgkin lymphoma, although the scientific evidence remains unclear.
Connections have also been made between various infections and non-Hodgkin lymphoma. “There is some evidence to show that some of the rarer types of non-Hodgkin lymphoma can be related back to the Epstein-Barr Virus or HIV,” says Pounds.
What are the symptoms?
For Sam Neill, neck lumps proved to be the tell-tale sign of the disease, and the most common sign of non-Hodgkin lymphoma is one or more painless swellings in the neck, armpit or groin, a sign of enlarged lymph nodes.
Pounds says that while symptoms tend to be quite vague, which is why a lot of patients are diagnosed at a relatively late stage, lumps tend to be smooth, rubbery, and not especially painful, but crucially they do not go away.
“Fatigue is also really common, and by that, not just being a bit tired but just unable to get out of bed,” she says. “And drenching night sweats, to the extent that you have to change your pyjamas and bedclothes. There are other signs that are more subtle, like getting infections or having a high temperature more often than normal. For some types of lymphoma, your skin can be a bit itchy, but there’s no rash.”
According to Cancer Research UK, the five-year survival rate for non-Hodgkin lymphoma is around 65 per cent and the 10-year survival rate is 55 per cent.
“Survival rates for the more common types are very good,” says Collins. “Overall, all the different types are treatable to a certain extent, and many forms are actually curable with the right therapy.”
How is non-Hodgkin lymphoma treated?
Because the lymphatic system exists throughout the body, patients are typically treated with various types of chemotherapy.
However, newer options are emerging for patients whose cancer has proven resistant to chemo. Glofitamab is an example of a bispecific antibody, a drug which consists of two arms, one of which latches on to a cancerous lymphoma cell and another that grasps on to a healthy white blood cell, before bringing the two together so that the cancerous cell can be destroyed.
“Bispecific antibodies seem to work well in both relapsed high-grade lymphoma and low-grade lymphoma,” says Collins. “These are also quite well-tolerated drugs, and we’re starting to see the first approvals in the UK, which is exciting.”
Another emerging treatment is a form of personalised medicine called CAR T-cell therapy, in which white blood cells called T cells are extracted from the patient and re-engineered to recognise that person’s cancer.
CAR T-cell therapy is expensive and as a result, the NHS previously limited access to patients with diffuse large B-cell lymphoma, who had already been through at least two other drug regimens without success.
However, in April 2023, it was announced that access to all patients with this form of the disease, who had already tried one other drug, without success.
Can it be prevented?
El-Sharkawi says that in order to more effectively understand how we might prevent non-Hodgkin lymphoma, we need to develop a better grasp of the risk factors for the disease.
“It’s not like lung cancer, where we understand that we need to reduce smoking rates to prevent the disease,” she says. “While we have some knowledge of the risks, there must be lots of other things causing it that we just don’t know about yet.”
To further improve survival rates, oncologists feel that there needs to be better diagnostic pathways to detect the disease at a much earlier stage.
It’s all about early diagnosis,” says Collins. “So rapid access to scans, rapid access to biopsies, because patients who are still relatively well are much easier to treat.”