About childhood lymphoma
Lymphoma originates in developing B lymphocytes and T-lymphocytes, which have undergone a malignant change. This means that they multiply without any proper order forming tumours, which are collections of cancer cells.
Over time, malignant lymphocytes (called lymphoma cells) crowd out normal lymphocytes and eventually the immune system becomes weakened and can no longer function properly.
There are 43 different sub-types of lymphoma currently recognised by the World Health Organization’s classification system for lymphoma. Five of these sub-types belong to a group of diseases called 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, which can be seen when examining the tumour cells under a microscope.
All other sub-types are commonly grouped together and called non-Hodgkin lymphoma (or B and T cell lymphomas). In children, lymphoma is one of the most common types of cancer seen, but few children overall are ever diagnosed with these diseases. Lymphomas in children tend to grow quickly but generally respond very well to treatment.
Significant advances are continually being made in the way we manage lymphomas. This means that with treatment, many children can now be cured. Many others who are treated remain disease-free and well for a long time.
How common is childhood lymphoma?
In Australia, around 30 children (0-14 years) are diagnosed with Hodgkin lymphoma and around 40 children (0-14 years) are diagnosed with non-Hodgkin lymphoma each year.
Who gets childhood lymphoma?
Hodgkin lymphoma can occur at any age but it is most common in adolescents and young adults. Hodgkin lymphoma is rarely seen in children less than five years old, and it occurs more frequently in males than in females.
Similarly to Hodgkin lymphoma, non-Hodgkin occurs more frequently in males. Non-Hodgkin lymphomas can occur at any age but they are more common in adults over the age of 50, who account for more than 70 per cent of all cases.
Causes of childhood lymphoma
In most cases the causes of childhood lymphoma remain 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 and it is thought the alterations in the immune system may play a role in some cases.
Children with a weakened immune system (immunosuppressed) either by a viral infection or as a result of drugs that affect the function of the immune system have an increased chance of developing lymphoma. Infection with Epstein-Barr virus (EBV), the virus that causes glandular fever, may be involved, particularly in people who are immunosuppressed.
In most cases, people who are diagnosed with lymphoma have no family history of the disease. However, there have been some cases 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 childhood lymphoma
Some children 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 for a different complaint. The most common symptom of 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 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 symptoms may include:
- recurrent fevers
- excessive sweating at night
- unintentional weight loss
- persistent fatigue and lack of energy
- generalised itching or a rash
- chronic cough/breathlessness (due to swollen lymph gland in chest)
- bowel changes/blockage (due to swollen glands in abdomen).
These symptoms are also seen in other illnesses such as viral infections. So, most people with these complaints do not have lymphoma. It is important to see your child’s doctor if your child has any symptoms that do not go away so that they can be examined and treated properly.
Diagnosis of childhood lymphoma
At a general examination a doctor will look and feel for any swelling of the glands in your child’s neck, armpits and groin. Your child’s abdomen and chest will also be examined for any signs of enlarged organs or fluid collection.
The doctor will also ask about any other symptoms your child might be having. At this stage some blood samples may be taken to check how well the bone marrow, liver and kidneys are functioning.
If the doctor suspects that your child might have lymphoma, a biopsy 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. To limit discomfort and distress, the biopsy is generally performed while the child is under a brief anaesthetic.
If the swollen gland is in the abdomen or pelvis, a surgical procedure called a laparoscopy may be 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 the abdomen or pelvis, and to take a biopsy if needed. This is performed under general anaesthetic. 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 your child has is important because it provides information on the most likely course of the disease and the best way to treat it.
Further tests
Once a diagnosis of lymphoma is made further tests need to be done to find out the extent to which the disease has spread in the body (the stage), and the effect it is having on important body organs. These include:
- blood tests: these are important because they provide a baseline set of results regarding the disease and your child’s general health. These results can be compared with later results to assess how well they are progressing.
- CT (computerized tomography): scans of chest, abdomen and pelvis are often used to assess the spread of lymphoma in the body
- MRI (magnetic resonance imaging): may be done when the doctor needs very clear pictures particularly of the brain and spinal cord, to see if these areas are affected by cancer
- PET (positron emission tomography): uses radioactive glucose, which is injected into a vein in the hand or arm. Using this technology areas of lymphoma are often more clearly visible than with other scans. It can also be used to assess response to treatment.
- 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 clinic or day procedure centre and is usually performed under a light anaesthetic or sedation given through a small drip in your child’s arm. The sample of bone marrow is examined in the laboratory to determine the number and type of cells present and the amount of haematopoiesis (blood forming) activity taking place there.
- CSF (cerebrospinal fluid) examination: a small sample of the CSF that surrounds your child’s brain and spinal cord is collected during a procedure called a lumbar puncture, and tested in the laboratory to check for the presence of lymphoma cells within the central nervous system (CNS).
Treatment of childhood lymphoma
Treatment varies depending on the exact type of lymphoma your child has, where it has spread in their body and how fast it is likely to grow. Their age and their general health are also taken into account.
In helping you to make the best treatment decision, your child’s doctor will consider all the information available including information gathered from hundreds of other people around the world who have had lymphoma and the details of your child’s particular situation.
Treatment for lymphoma usually involves chemotherapy or radiotherapy and surgery. Peripheral blood stem cell transplantation can be used when the lymphoma has come back (relapsed) or is at high risk of relapse, and where it doesn’t respond well to standard (conventional) treatment.
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 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 child’s arm or hand, or in tablet or liquid form (orally). Your child’s doctor may recommend that your child 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.
Length of treatment cycles is dependent on the type of lymphoma your child has and their response to treatment. Those with low-stage lymphoma are generally treated with a less intensive combination of chemotherapy drugs. Children with high-stage disease are treated with more intensive chemotherapy.
Hodgkin and B-cell lymphomas are generally treated over a few months to a year while treatment for T-cell lymphoma can be around two years. If lymphoma cells have been found in your child’s brain or spinal column, intrathecal chemotherapy will be given. This is chemotherapy injected directly into the spinal fluid via a needle in a procedure called a lumbar puncture. A light anaesthetic or mild sedation is given to your child for this treatment.
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 weekday (Monday to Friday) over a few weeks in the radiotherapy department of the hospital. Your child does not see or feel anything during the actual radiotherapy treatment. Radiotherapy can be conducted as an outpatient.
Before radiotherapy begins, the radiotherapist (doctor who specialises in treating people with radiotherapy) will carefully calculate the correct dose of radiation therapy for your child. The areas of their body that need to be treated will be marked with tiny ink dots on their 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 your child is having radiotherapy they will usually lie on a table underneath the radiotherapy machine which delivers the planned dose of radiation. Important structures like their 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 your child will not see or feel anything during the actual treatment. They will however need to stay perfectly still for a few minutes while the treatment is taking place.
Prognosis
It is important to remember that the prognosis for children with lymphoma is generally good and the majority recover completely.
Your treating specialist is the best person to discuss your child’s prognosis and treatment options with as your child’s recovery is dependent on what type of lymphoma they have, the extent of their disease and any previous medical history.
Side effects of childhood 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 to the doctor or nurse. In most cases they can be treated and are reversible. Information about the specific side effects of your child’s treatment should be discussed with your treating doctor.
When to contact your doctor or hospital
As a general rule, while your child is having treatment you will need to contact your doctor or hospital immediately if they have any of the following:
- a temperature of 38oC or over (even if it returns to normal) and/or an episode of uncontrolled shivering (a rigor)
- bleeding or bruising, for example blood in their urine, faeces, sputum, bleeding gums or a persistent nose bleed
- nausea or vomiting that prevents them from eating or drinking or taking their 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 they cut or otherwise injure themselves
- if you notice pain, swelling, redness or pus anywhere on their body.
What are the side effects of childhood lymphoma treatment?
Side effects of chemotherapy and radiotherapy
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 depend 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 the 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, the blood count (the number of white cells, platelets and red cells circulating in the blood) will generally fall within a week of treatment. The length of time it takes for the bone marrow and blood counts to recover mainly depends on the type of chemotherapy given.
When the platelet count is very low (thrombocytopenic) your child can bruise and bleed more easily. During this time it is helpful to avoid sharp objects in their mouth such as chop bones or potato chips as these can cut their gums. Using a soft toothbrush also helps to protect their gums. In many cases a transfusion of platelets is given to reduce the risk of bleeding until the platelet count recovers.
If the red blood cell count and haemoglobin levels drop they will probably become anaemic. When a child is anaemic they feel more tired and lethargic than usual. If their haemoglobin level is very low, the doctor may prescribe a blood transfusion.
Risk of Infection
The point at which the white blood cell count is at its lowest is called the nadir. This is usually expected 10 to 14 days after having chemotherapy. During this time your child will be at a higher risk of developing an infection. At this stage they will also be neutropenic, which means that their neutrophil count is low.
Neutrophils are important white blood cells that help us to fight infection. While your child’s 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 (e.g. colds, flu, chicken pox) and your child only eating food that has been properly prepared and cooked. If your child does develop an infection they may experience a fever, which may or may not be accompanied by an episode of shivering where they shake uncontrollably. Infections while they are neutropenic can be quite serious and need to be treated with antibiotics as soon as possible.
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. Your child will be given anti-sickness drugs before and for a few days after their chemotherapy treatment. Be sure to tell the nurses and doctors if the anti-emetics are not working for your child. 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 your child is feeling sick.
Changes in taste and smell
Both chemotherapy and radiation therapy can cause changes to the sense of taste and smell. This is usually temporary but in some cases it lasts up to several months. There are lots of reasons why children may not feel like eating much, especially while they are having treatment or are in hospital. Allowing your child to eat when they are hungry, which often means snacking in between meal times, and offering them nutritious snacks and drinks throughout the day can be helpful.
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 their blood count recovers, usually a couple of weeks later. During this time the 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. Always check your child’s temperature before giving them pain medication as this drug can ‘mask’ signs of infection (a raised temperature). If the pain becomes more severe, stronger pain killers might be needed. It is important to keep your child’s mouth as clean as possible while they are having treatment to help prevent infection. Avoid commercial mouthwashes as these are often too strong or they may contain alcohol, which will hurt their mouth.
Bowel changes
Chemotherapy can cause some damage to the lining of the bowel wall. This can lead to cramping, wind, abdominal swelling and diarrhoea. Be sure to tell the nurses and doctors if your child experiences any of these symptoms. If they develop diarrhoea, a specimen will be required to ensure that the diarrhoea is not the result of an infection. After this they will be given some medication to help stop the diarrhoea and/or the discomfort. It is also important to tell the nurse or doctor if they are constipated or feeling any discomfort or tenderness around their anus while they are trying to move their bowels. They may need a gentle laxative to help soften their bowel motion.
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.
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 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 your child is losing their hair.
Your child will need to avoid direct sunlight on their exposed head (get them to wear a hat) because chemotherapy (and radiotherapy) makes their skin even more vulnerable to the damaging effects of the sun (i.e. sunburn and skin cancers). Remember that without hair their head can get quite cold so a beanie might be useful. Hair can also be lost from their eyebrows, eyelashes, arms and legs.
Many young children are not worried by losing their hair and are happy to wear hats, scarves or bandanas. Older children and teenagers however, are often more concerned about the effects of hair loss and other changes to their appearance. They are often encouraged to get a wig; while they may never wear it, having the wig may give them the confidence to participate in everyday activities, particularly those involving friends.
Fatigue
Most children experience some degree of tiredness in the days and weeks following chemotherapy and radiotherapy. Getting plenty of rest and a little light exercise each day may help to make your child feel better during this time. It is important for them to rest when they are tired.
Fertility
Most children who are treated for lymphoma will grow up and be able to have normal, healthy babies. For others, treatment may cause a reduction in their fertility and their ability to have children in the future. This may depend on the age of the child when they were treated and the type of treatment they received.
In boys, sperm production may be impaired for a while following chemotherapy but it is important to realise that production of new sperm may become normal again in the future.
In girls chemotherapy and radiotherapy can cause varying degrees of damage to the normal functioning of the ovaries. This will depend on the age of the child and the dose of radiotherapy or chemotherapy given. In some cases this leads to menopause earlier than expected. The onset of puberty can also be affected and some children may require hormonal supplements to ensure normal sexual development.
Body image, sexuality and sexual activity
Hair loss, skin changes and fatigue can all interfere with how your child feels about and sees themselves. If you have older children who are sexually active, it is usually recommended that they do not become pregnant as some of the treatments given might harm the developing baby; therefore a suitable form of contraception should be used.
Seizures
Intrathecal (IT) therapy is rarely associated with seizures, otherwise known as fitting. If your child experiences a seizure, or if the doctor feels they may be at risk of having a seizure, they will prescribe special medication to help to prevent this from happening.
Side effects of cortico-steroids
Side effects of cortico-steroids depend largely on how long they are used for and the dose given. Again, children respond differently. An increased appetite, fluid retention and weight gain and the classic ‘moon-shaped’ face and swollen belly are common side effects of these drugs. Some children find it more difficult to get to sleep at night and to stay asleep and some night sedation may be required. Mood swings, anxiety, restlessness and nightmares are also common side effects of steroid therapy. A child’s moods and behaviours can be challenging while they are receiving steroids.
While accepting that some allowances need to be made, maintaining your normal parenting strategies is important during this time. Being consistent and setting limits on your child’s behaviour can help to make them feel more secure. It can also help to prevent unpleasant longer-term behavioural problems, which can cause considerable stress within any family.
Long-term use of steroid therapy may cause other effects such as fluid retention, an increased susceptibility to infections, or osteoporosis, where the bones may become weak and brittle. However, these effects are not common as most children with lymphoma do not require prolonged steroid therapy.
Caring for someone with childhood lymphoma
We have a range of information and resources that may help when you are caring for someone with childhood lymphoma.
