Non-Hodgkin lymphoma

About non-Hodgkin lymphoma

The many different sub types of non-Hodgkin lymphoma are broadly divided into the following two main groups:

• B-cell lymphomas – arising from developing B-cells
• T-cell lymphomas – arising from developing T-cells.

B and T-cell lymphomas are cancers of the lymphatic system. The lymphatic system forms part of the immune system. It contains specialised white blood cells called lymphocytesthat help protect the body from infection and disease. Lymphomas arise when developing B- and T- lymphocytes undergo a malignant change, and multiply in an uncontrolled way. These abnormal lymphocytes, called lymphoma cells, form collections of cancer cells called tumours, in lymph nodes (glands) and other parts of the body.

How common is non-Hodgkin lymphoma?

Overall, it represents the sixth most common type of cancer in men, and the fifth most common type of cancer in women.

Who gets non-Hodgkin lymphoma?

Lymphomas can occur at any age but they are more common in adults over the age of 50. Lymphomas occur more frequently in men than in women. In children non-Hodgkin lymphoma and leukaemia are some of the most common types of cancer seen, but this number is far fewer than in the adult population. Lymphomas in children tend to grow quickly and they are often curable.

Causes of non-Hodgkin lymphoma

In most cases the exact cause of lymphoma remains unknown but they are thought to result from damage to one or more of the genes that normally control the development of blood cells. Research is going on all the time into possible causes of this damage. In most cases people who are diagnosed with lymphoma have no family history of the disease. Like many cancers, damage to special proteins that control the growth and division of cells may play a role in the development of lymphoma. The following are factors which may put some people at a higher risk of developing lymphoma:

• immunosuppression – a small percentage of lymphomas occur in people whose immune system has been weakened either by a viral infection or as a result of drugs that affect the function of the immune system
• infection – particularly in people with immunosuppression, viruses such as the Epstein-Barr virus or the human T-cell leukaemia/lymphoma virus may damage developing lymphocytes
• chemicals – some evidence suggest that people exposed to high concentrations of agricultural chemicals may have a higher risk of developing lymphoma
• lifestyle – lifestyle factors including smoking and obesity can increase the risk of developing lymphoma.

Symptoms of non-Hodgkin lymphoma

Some people do not have any symptoms when they are first diagnosed with lymphoma. In these cases the disease may be picked up by accident, for example during a routine chest x-ray. Lymphoma commonly presents as a firm, usually painless swelling of a lymph node (swollen glands), usually in the neck, under the arms or in the groin. It is important to remember that most people who go to their doctor with enlarged lymph nodes do not have lymphoma. Swollen glands often result from an infection, for example a sore throat. In this case the glands in the neck are usually swollen and painful. Other non-Hodgkin lymphoma symptoms may include:

• regular and frequent fevers
• excessive sweating, usually at night
• unintentional weight loss
• persistent fatigue and lack of energy
• generalised itching.

Sometimes lymphoma starts in the lymph nodes in deeper parts of the body like those found in the abdomen (causing bloating), or the lymph nodes in the chest (causing coughing, discomfort in the chest and difficulty breathing). When it is first diagnosed, it is common for lymphoma to be found in several different sites in the body at once. It can spread to any organ and may involve the spleen, liver, brain and spinal cord (central nervous system) and bone marrow. Some of these symptoms may also be seen in other illnesses, including viral infections. It is important to see your doctor if you have any symptoms that do not go away so that you can be examined and treated properly.

Diagnosis of non-Hodgkin lymphoma

General examination and blood samples

At a general examination a doctor will look and feel for any swelling of the glands in your neck, armpits and groin. Your abdomen and chest will also be examined for any signs of enlarged organs or fluid collection. The doctor will also ask you about any other symptoms you might have. At this stage some blood samples may be taken to check how well your bone marrow, liver and kidneys are functioning. Knowing the exact type of lymphoma you have is important because it provides information on the most likely course of your disease and the best way to treat it.

Biopsy

If the doctor suspects that you might have lymphoma, a biopsy will need to be done to help confirm the diagnosis. A biopsy involves removing a lymph node or a sample of tissue from a suspicious lump for examination in the laboratory. There are different types of biopsies. A core or fine needle biopsy involves inserting a needle into a lymph node or lump and removing a sample of tissue. This is usually done under local anaesthetic, while you are awake. If the affected lymph node is deep inside the body the biopsy may be done with the help of ultrasound or specialised x-ray (imaging) guidance.

Laparoscopy

If the swollen gland is in the abdomen or pelvis, a surgical procedure called a laparoscopy is performed in which a narrow tube with a microscope attached is inserted through a small cut in the skin. It allows the surgeon to see the inside of your abdomen or pelvis, and to take a biopsy if needed. This is done under general anaesthetic and you will have a few stitches afterwards. Once the tissue samples are removed they are examined in the laboratory by a pathologist. It can take a few days for the final results of the biopsy to come through.

Further tests after diagnosis

Once a diagnosis of non-Hodgkin lymphoma is made further tests need to be done to find out the extent to which the disease has spread in your body (the stage), and the effect it is having on important body organs. These tests include:

Blood tests

Blood tests are important because they provide a baseline set of results regarding your disease and general health. These results can be compared with later results to assess how well you are progressing.

CT scans (computerised tomography)

A CT scan of the chest, abdomen and pelvis is often used to assess the spread of lymphoma in the body.

MRI (magnetic resonance imaging)

An MRI scan is done when the doctor needs very clear pictures of the brain and spinal cord, to see if these areas are affected by cancer.

Gallium scan

This is an imaging technique that involves the use of radioactive gallium (a type of metal in a liquid form). The gallium is injected into a vein in your hand or arm at the nuclear medicine department of the hospital. You leave the unit and return 24-48 hours later by which time your body is scanned from several different angles, to detect the presence of lymphoma.

PET scanning (positron emission tomography)

This scan involves radioactive glucose being injected into a vein in your hand or arm. Using this technology, slow-growing lymphomas can be distinguished from fast-growing ones because of the rate at which the glucose is used by the different cells.

Bone marrow biopsy

A sample of bone marrow is taken, usually from the back of the hip bone, and sent to the laboratory for examination under the microscope. The bone marrow biopsy may be done in the haematologist’s rooms, clinic or day procedure centre and is usually performed under local anaesthesia with sedation given either by tablet or through a small drip in your arm. The sample of bone marrow is examined in the laboratory to determine the number and type of cells present and the amount of haemopoiesis (blood forming) activity taking place there.

Treatment of non-Hodgkin lymphoma

Treatment varies depending on the exact type of B or T-cell lymphoma you have, where it has spread in your body and how fast it is likely to grow. Your age and your general health are also taken into account. When deciding how to treat your lymphoma it is important to know how fast it is likely to grow and cause problems in your body. This is called the grade of your lymphoma.

Some types of lymphoma grow slowly, cause few symptoms and may not need to be treated urgently. These are known as indolent lymphomas (also called low-grade lymphomas). Others grow more quickly, cause more severe symptoms and generally need to be treated soon after they are diagnosed. These are known as aggressive lymphomas (also called intermediate-grade and high-grade lymphomas).

Low-grade lymphomas (indolent)

Many low-grade lymphomas take years to grow, cause few if any symptoms and do not necessarily need to be treated initially. In these cases the doctor may recommend regular checkups to carefully monitor your health. If the disease is limited to a small group of lymph nodes, radiotherapy alone may be able to cure some low-grade lymphomas, or control them for a long time.

If the low-grade lymphoma is more widespread in the body, the decision to treat the disease or not depends on where the lymphoma has spread to, how large the spots of lymphoma are, and your general condition. In some situations chemotherapy is given, either in tablet form or intravenously. This is usually very effective and puts many patients into a remission that lasts a variable length of time. In some cases, low-grade lymphomas develop over time into more aggressive or high grade lymphomas requiring more aggressive treatment.

Intermediate and high-grade lymphomas

Intermediate-grade and high-grade lymphomas grow quickly and treatment is needed when they are diagnosed. If these diseases respond well to chemotherapy, they can sometimes be cured. Chemotherapy is usually combined with rituximab in B-cell lymphomas to give the best results.

Relapsed disease

Finding out that your disease has come back (relapsed) or is resistant to standard treatment can be devastating. If your disease does relapse there are usually ways of getting it back under control. These might involve using more combination chemotherapy, or using more intensive or high dose chemotherapy followed by a bone marrow or peripheral blood stem cell transplant.

Palliative care

If a decision is made not to continue with anti-cancer treatment (chemotherapy and radiotherapy) for your lymphoma there are still many things that can be done to help you to stay as healthy and comfortable as possible for some time. Palliative care is aimed at relieving any symptoms or pain you might be experiencing as a result of your disease or its treatment, rather than trying to cure or control it.

Types of treatment

Treatment for lymphomas usually involves chemotherapy or radiotherapy and surgery. Peripheral blood stem cell transplantation, steroid therapy and biotherapies may also be used.

Radiotherapy

Radiotherapy uses high energy x-rays to kill cancer cells and shrink cancers. Radiotherapy is usually given in small doses (also known as fractions) each weekday (Monday to Friday) over a few weeks in the radiotherapy department of a hospital.  You do not see or feel anything during the actual radiotherapy treatment. You will, however, need to stay perfectly still for a few minutes while the treatment is taking place.

Before you start radiotherapy, the radiotherapist (doctor who specialises in treating people with radiotherapy) will carefully calculate the correct dose of radiation therapy for you. The areas of your body that need to be treated will be marked with tiny ink dots on your skin using a special pen. Radiotherapy is usually given in small doses (also known as fractions) each week day (Monday to Friday) over a few weeks in the radiotherapy department of the hospital.

When you are having radiotherapy you usually lie on a table underneath the radiotherapy machine which delivers the planned dose of radiation. Important structures like your heart and lungs are shielded as much as possible to ensure that they are not affected by the treatment given. Radiotherapy is painless. In fact you do not see or feel anything during the actual treatment. You will however need to stay perfectly still for a few minutes while the treatment is taking place. You might like to bring along some music to help you relax.

Chemotherapy

Chemotherapy literally means therapy with chemicals. Many chemotherapy drugs are also called cytotoxics (cell toxic) because they kill cells, especially ones that multiply quickly like cancer cells. Chemotherapy is usually given as a combination of drugs, in several cycles (or courses) of treatment with a rest period of a few weeks in between each cycle. Chemotherapy may be given in either tablet form or intravenously, into a vein in your hand or arm, or through a special line called a central venous catheter inserted before you start treatment.

A typical chemotherapy regime for lymphoma might involve around six cycles of a combination of drugs, given over a period of several months. Improved results have been achieved by combining chemotherapy with monoclonal antibody therapy which is specifically engineered to target lymphoma cells. Once antibodies bind to these cells, a patient’s own immune system is then able to recognise these cancer cells as foreign and kill them.

Stem cell transplant

Occasionally, a stem cell transplant is given for non-Hodgkin lymphoma, providing some people with a better chance of cure or long-term control of their disease. It is generally only suitable in some situations where the lymphoma has come back (relapsed) or is at a high risk of relapse, and where it doesn’t respond well to standard (conventional) treatment.

Side effects of non-Hodgkin lymphoma treatment

All treatments can cause side effects. However, the type and severity will vary between individuals, depending on the type of treatment used and how an individual responds to it. In general, more intensive treatment is associated with more severe side effects. It is important to report any symptoms you are having to your doctor or nurse. In most cases they can be treated and are reversible.

When to contact your doctor or hospital

As a general rule, while you are having treatment you will need to contact your doctor or hospital immediately if you have any of the following:

• a temperature of 38^oC or over (even if it returns to normal) and/or an episode of uncontrolled shivering (a rigor)
• bleeding or bruising, for example blood in your urine, faeces, sputum, bleeding gums or a persistent nose bleed
• nausea or vomiting that prevents you from eating or drinking or taking your normal medications
• severe diarrhoea, stomach cramps or constipation
• coughing or shortness of breath
• a new rash, reddening of the skin, itching
• a persistent headache
• a new pain or soreness anywhere
• if you cut or otherwise injure yourself
• if you notice pain, swelling, redness or pus anywhere on your body.

What are the side effects of non-Hodgkin lymphoma treatment?

Radiotherapy can cause similar side effects to those caused by chemotherapy including nausea and vomiting, hair loss and fatigue. In general, the type of side effects seen with radiotherapy depends on the area of the body which has been treated. Skin reactions are common.

Chemotherapy kills cells that multiply quickly, such as lymphoma cells. It also causes damage to fast-growing normal cells, including hair cells and cells that make up the tissues in your mouth, gut and bone marrow. The side effects of chemotherapy occur as a result of this damage.

The side effects of radiotherapy and chemotherapy can include:

Effects on the bone marrow

Chemotherapy affects the bone marrow’s ability to produce adequate numbers of blood cells. As a result, your blood count (the number of white cells, platelets and red cells circulating in your blood) will generally fall within a week of treatment. The length of time it takes for your bone marrow and blood counts to recover mainly depends on the type of chemotherapy given.

When your platelet count is very low (thrombocytopenic) you can bruise and bleed more easily. In many cases a transfusion of platelets is given to reduce the risk of bleeding until the platelet count recovers.

If your red blood cell count and haemoglobin levels drop you will probably become anaemic. When you are anaemic you feel more tired and lethargic than usual. If your haemoglobin level is very low, your doctor may prescribe a blood transfusion.

Parotitis

Associated with radiotherapy, parotitis is an inflammation of the saliva-producing glands in the mouth, which can occur if these glands are within the treatment field used. These include the parotid or submandibular glands, which are situated at the top of the jaw line, in front of the ears. Parotitis causes dryness of the mouth and jaw pain, which usually settles down within a few days, once the inflammation subsides.

Risk of infection

The point at which your white blood cell count is at its lowest is called the nadir. This is usually expected 10-14 days after having your chemotherapy. During this time you will be at a higher risk of developing an infection. At this stage you will also be neutropenic, which means that your neutrophil count is low.

Neutrophils are important white blood cells that help us to fight infection. While your white blood cell count is low you should take sensible precautions to help prevent infection. These include avoiding crowds, avoiding close contact with people with infections who are contagious (for example colds, flu, chicken pox) and only eating food that has been properly prepared and cooked. If you do develop an infection you may experience a fever, which may or may not be accompanied by an episode of shivering where you shake uncontrollably. Infections while you are neutropenic can be quite serious and need to be treated with antibiotics as soon as possible. Sometimes your doctor may decide to use a drug  to help the recovery of your neutrophil count. This drug works by stimulating the bone marrow to increase the production of neutrophils and is usually given as an injection under the skin (subcutaneous).

Nausea and vomiting

Nausea and vomiting are often associated with chemotherapy and some forms of radiotherapy. These days however, thanks to significant improvements in anti-sickness (anti-emetic) drugs, nausea and vomiting are generally very well controlled. You will be given anti-sickness drugs before and for a few days after your chemotherapy treatment. Be sure to tell the nurses and doctors if the anti-emetics are not working for you and you still feel sick. Some people find that eating smaller meals more frequently during the day, rather than a few large meals, helps to reduce nausea and vomiting. Many find that eating cool or cold food is more palatable, for example jelly or custard. Drinking ginger ale or soda water and eating dry toast may also help if you are feeling sick.

Changes in taste and smell

Both chemotherapy and radiation therapy can cause changes to your sense of taste and smell. This is usually temporary but in some cases it lasts up to several months.

Mucositis

Mucositis, or inflammation of the lining of the mouth, throat or gut is a common and uncomfortable side effect of chemotherapy and some forms of radiotherapy. It usually starts about a week after the treatment has finished and goes away once your blood count recovers, usually a couple of weeks later. During this time your mouth and throat could get quite sore. Soluble pain medication and other topical drugs (ones which can be applied to the sore area) can help. If the pain becomes more severe, stronger pain killers might be needed. It is important to keep your mouth as clean as possible while you are having treatment to help prevent infection. Avoid commercial mouthwashes, as these are often too strong or may contain alcohol, which will hurt your mouth.

Bowel changes

Chemotherapy can cause some damage to the lining of your bowel wall. This can lead to cramping, wind, abdominal swelling and diarrhoea. Be sure to tell the nurses and doctors if you experience any of these symptoms. If you are constipated or if you are feeling any discomfort or tenderness around your anus when you are trying to move your bowels, you may need a gentle laxative to help soften your bowel motions.

Hair loss

Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. It is, however, usually only temporary. The hair can start to fall out within a couple of weeks of treatment and tends to grow back three to six months later. Avoiding using heat or chemicals and only using a soft hairbrush and a mild baby shampoo can help reduce the itchiness and scalp tenderness which can occur while you are losing your hair. You need to avoid direct sunlight on your exposed head (wear a hat) because chemotherapy and radiotherapy makes your skin even more vulnerable to the damaging effects of the sun (like sunburn and skin cancers). Remember that without your hair your head can get quite cold so a beanie might be useful. Hair can also be lost from your eyebrows, eyelashes, arms and legs.

Fatigue

Most people experience some degree of tiredness in the days and weeks following chemotherapy and radiotherapy. Getting plenty of rest and doing a little light exercise each day may help to make you feel better during this time. It is important to listen to your body and rest when you are tired.

Fertility

Some types of chemotherapy and radiotherapy may cause a temporary or permanent reduction in your fertility. If possible, before you commence treatment you should discuss any questions or concerns you might have regarding your future fertility with your doctor. In women, some types of chemotherapy and radiotherapy can cause varying degrees of damage to the normal functioning of the ovaries.

In men, sperm production can be impaired for a while but the production of new sperm may become normal again in the future. There are some options for preserving your fertility, if necessary, while you are having treatment for lymphoma.

Early menopause

Some cancer treatments can affect the normal functioning of the ovaries. This can sometimes lead to infertility and an earlier than expected onset of menopause, even at a young age. The onset of menopause in these circumstances can be sudden and, understandably, very distressing. Hormone changes can lead to many of the classic symptoms of menopause. Some women experience decreased sexual drive, anxiety and even depressive symptoms during this time. It is important that you discuss any changes to your periods with your doctor or nurse.

Body image, sexuality and sexual activity

Hair loss, skin changes and fatigue can all interfere with how you feel about yourself as a man or a woman and as a ‘sexual being’. During treatment you may experience a decrease in libido and it may take some time for things to return to ‘normal’. It’s perfectly reasonable and safe to have sex while you are on treatment or shortly afterwards, but there are some precautions you need to take. It is usually recommended that you or your partner do not become pregnant as some of the treatments given might harm the developing baby. As such, you need to ensure that you or your partner use a suitable form of contraception. Condoms are also necessary in the seven days following a treatment session, to ensure that your partner is not exposed to any residual drugs. Partners are sometimes afraid that sex might in some way harm the patient. This is not likely as long as the partner is free from any infections and the sex is relatively gentle.

Caring for someone with non-Hodgkin lymphoma

We have a range of information and resources that may help when you are caring for someone with non-Hodgkin lymphoma.