Hodgkin lymphoma

About Hodgkin lymphoma

Hodgkin lymphoma is distinguished from all other types of lymphoma because of the presence  of a special kind of cancer cell called a Reed-Sternberg cell. When it is first diagnosed, Hodgkin lymphoma may affect a single lymph node or a group of lymph nodes or it may affect another part of the body such as the spleen, liver or bone marrow.

In more advanced stages, Hodgkin lymphoma can spread via the lymphatic system and the blood to almost any part of the body. With treatment, most people with Hodgkin lymphoma can be cured. This is especially true for younger patients who are diagnosed with early stage disease. Advanced stage Hodgkin lymphoma can also be treated very successfully.

What are the sub types of Hodgkin lymphoma?

The five different types of Hodgkin lymphoma are distinguished from each other by the appearance of the lymphoma cells under the microscope.

• Nodular sclerosing: more commonly seen in young adults and usually involves the lymph glands of the neck and chest.
• Mixed cellularity: more commonly seen in older people (over 50 years).
• Lymphocyte-rich: a rare sub-type with the most favourable outcome of all the Hodgkin lymphomas. This sub-type involves very few Reed-Sternberg cells making it challenging to diagnose correctly.
• Lymphocyte depleted: this disease tends to be more widespread at diagnosis.
• Nodular lymphocyte predominant: tends to be slow growing and responds well to treatment with radiotherapy and chemotherapy.

How common is Hodgkin lymphoma?

Each year in Australia, around 600 people are diagnosed with Hodgkin lymphoma. Overall, Hodgkin lymphoma is a rare disease accounting for around 0.5% of all cancers and around 10% of all lymphomas diagnosed.

Who gets Hodgkin lymphoma?

Hodgkin lymphoma can occur at any age but it is most common in adolescents and young adults, with over a third of all cases diagnosed between the ages of 15 and 30. Around 30 children (0-14 years) are diagnosed with Hodgkin lymphoma each year.

Hodgkin lymphoma occurs more frequently in males than in females.

Causes of Hodgkin lymphoma

In most cases the causes of Hodgkin lymphoma remains unknown. We do know that it is not contagious and like many cancers, damage to special proteins which normally control the growth and division of cells may play a role in its development. Research is going on all the time into possible causes of this damage.

People with a weakened immune system (immunosuppressed) due to an inherited immune deficiency disease, HIV infection, or drugs taken to prevent rejection of a transplanted organ, all have an increased chance of developing lymphoma. Infection with Epstein-Barr virus (EBV), the virus that causes glandular fever, may put some people at a higher risk of developing of Hodgkin lymphoma.

In most cases, people who are diagnosed with Hodgkin lymphoma have no family history of the disease. There have been some cases, however, where a brother or sister also develops Hodgkin lymphoma, suggesting a rare family genetic link to the disease. As this is very rare, it is not recommended that families undergo testing for the disease.

Symptoms of Hodgkin lymphoma

Some people do not have any symptoms when they are first diagnosed with Hodgkin lymphoma. In these cases the disease may be picked up by accident, for example during a routine chest x-ray. The most common symptom of Hodgkin lymphoma is 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 Hodgkin 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 Hodgkin lymphoma symptoms may include:

• recurrent fevers
• excessive sweating at night
• unintentional weight loss
• persistent fatigue and lack of energy
• generalised itching or a rash.

These symptoms are also seen in other illnesses such as viral infections. Most people with these complaints do not have Hodgkin lymphoma. However, 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 Hodgkin lymphoma

General examination and blood tests

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. If the doctor suspects that you might have Hodgkin lymphoma, further testing will need to be done to help confirm the diagnosis.

Biopsy

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. You will need a general anaesthetic for this and you will have a few stitches afterwards.

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. 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.

Further tests after diagnosis

Once a diagnosis of 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)

CT scans of the chest, abdomen and pelvis are often used to assess the spread of lymphoma in the body.

MRI (magnetic resonance imaging)

An MRI may be 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)

A PET scan involves the painless injection of a small amount of a ‘positron-emitting’ radioactive material (called a radiopharmaceutical). Images of the body are then taken using a PET scanner. The camera detects emissions coming from the injected radiopharmaceutical, and the computer attached to the camera creates two and three-dimensional images of the area being examined.

Bone marrow biopsy

If the results of your blood tests suggest that you might have lymphoma, a bone marrow biopsy may be required to help confirm the diagnosis. A bone marrow biopsy involves taking a sample of bone marrow (usually from the back of the hip bone) and sending it 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 Hodgkin lymphoma

Treatment depends mainly on the stage, or extent of disease in your body. Other factors that are considered include the exact type of Hodgkin lymphoma you have, your age and general health. These days, most people treated for Hodgkin lymphoma can be cured. Many others who are treated remain disease-free and well for a long time. Treatment usually involves chemotherapy or radiotherapy or a combination of both. In selected cases, a stem cell transplant (also called a peripheral blood stem cell or bone marrow transplant) may be used.

Early-stage disease

The great majority of people with early-stage Hodgkin lymphoma (stages 1 and 2) are cured. There are often several options available for your treatment, which your doctor should discuss with you. Early-stage disease is usually treated with a combination of chemotherapy and radiotherapy. The decision regarding which of these options should be used will depend on your disease stage and which side effects of treatment are important to you.

Advanced-stage disease

Advanced-stage disease (stages 3 and 4) is usually treated with a longer course of chemotherapy. In most patients, the chemotherapy will continue for six months or even longer. Occasionally radiotherapy is also used in this stage.

Relapsed and resistant disease

Finding out that your disease has come back or relapsed can be devastating. If your disease does relapse there are usually ways of trying to get it back under control. These might involve using more chemotherapy, which might be a different type to before, or using more intensive or high-dose chemotherapy followed by a 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.

Radiotherapy

Radiotherapy is a form of local therapy, because it only destroys cancer cells in the treated area (radiation field). It is commonly used to treat disease that is confined to one area of the body. 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 week day (Monday to Friday) over a few weeks in the radiotherapy department of a hospital.

Before radiotherapy begins, 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.

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 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. A typical chemotherapy regime for Hodgkin lymphoma might involve around six cycles of a combination of drugs, given over a period of six months. There are many different ways of giving chemotherapy. It may be given through a vein (intravenously or IV), usually in your arm or hand, or in tablet form (orally).

If you are having several cycles of intravenous chemotherapy, your doctor may recommend that you have a central venous catheter (also called a central line) inserted. Once in place, chemotherapy and any other IV drugs can be given through the line and blood tests can usually be taken from the line. There are several different kinds of central lines used. Some are intended for short-term use while others remain in place for months and even years.

In most cases you do not need to be admitted for chemotherapy. It is usually given in the outpatient department of the hospital. Sometimes, however, depending on the type of chemotherapy being given and your general health, you may need to be admitted to the ward for a short while.

Stem cell transplant

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

Side effects of Hodgkin lymphoma treatment

All treatments can cause side effects. The type and severity of these side effects 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.

Radiotherapy and chemotherapy side effects

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 that has been treated. Skin reactions are common.

Chemotherapy kills cells that multiply quickly, such as lymphoma cells. It also causes damage to fast-growing normalcells, 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. These 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. During this time it is helpful to avoid sharp objects in your mouth such as chop bones or potato chips as these can cut your gums. Using a soft toothbrush also helps to protect your gums. 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.

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 to 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 contagious infections (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 that 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 they 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 develop diarrhoea, a specimen will be required from you to ensure that the diarrhoea is not the result of an infection. After this you will be given some medication to help stop the diarrhoea and/or the discomfort you may be feeling. It is also important to tell the nurse or doctor 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 motion.

Hair loss

Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. However, it is usually only temporary. The hair starts to fall out within a couple of weeks of treatment and tends to grow back three to six months later. Avoiding the use of heat or chemicals and only using a soft hairbrush and a mild baby shampoo can help reduce the itchiness and scalp tenderness that 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 (i.e. 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 maycause a temporary or permanent reduction in your fertility. It is very important that you discuss any questions or concerns you might have regarding your future fertility with your doctor, if possible beforeyou commence treatment. 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 a 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 is 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 uses a suitable form of contraception. 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. Finally, if you are experiencing vaginal dryness, a lubricant can be helpful.

Caring for someone with Hodgkin lymphoma

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