We're here to talk | 24-hours a day
08088 010 444FREE from landlines & most major mobile networks
Providing support to anyone affected by blood cancer
Childhood acute lymphoblastic leukaemia (ALL) is a rare blood cancer, which affects the lymphocytic (antibody producing) white blood cells that are produced by the bone marrow. It is considered rare as it affects about five in every 100,000 children per year.
This information describes diagnosis and treatment of common ALL in children aged between 1 and 15 years old. If your child has a different type of ALL, or is older or younger, the differences in treatment will be explained to you by your child’s doctor.
When someone has ALL, there is an excess number of abnormal lymphocytes in the circulating blood which are unable to help fight infection in the body. The marrow is unable to make enough normal blood cells and this may lead to a set of debilitating symptoms.
There are several different types of lymphocyte, the main types are called B and T cells and have different jobs in the immune system. Most cases of childhood ALL are a type called precursor B-cell ALL, also sometimes called common-ALL.
Acute means that that the cancer comes on quickly and, if left untreated, progress quickly, Lymphoblastic refers to the presence of large numbers of lymphoblasts which are immature white blood cells.
ALL is the most common cancer type in children and it usually responds to treatment very well. About nine out of ten (90%) of patients with this form of leukaemia being long-term survivors.
In almost all cases of childhood ALL there is no apparent cause. The only thing known to increase the risk of ALL is exposure to high levels of radiation, but this is unlikely to account for very few, if any, cases in the UK.
Several things have been suggested causes, including living near power lines or nuclear power plants, mobile phones or phone masts or chemicals. However, studies have not confirmed any of these as a cause of childhood ALL.
Some evidence suggests that timing and pattern of exposure to infections may affect the risk of developing childhood ALL, but this is not well understood.
ALL cannot be caught from someone and it cannot be passed from parent to child.
In a child with ALL, the marrow contains large numbers of lymphoblasts and other immature blood cells. The production of normal blood cells (red cells, white cells and platelets) is reduced because of leukaemia. The blood will almost always contain large numbers of lymphoblasts, which usually makes diagnosis obvious. In rare cases, the blood count may be normal or there may be fewer than normal blood cells.
ALL patients have lower than normal numbers of red blood cells (anaemia), white blood cells (neutropenia) and/or platelets (thrombocytopenia). When all types of blood cell are lower than normal, this is called pancytopenia. These changes lead to most symptoms of ALL.
The most common symptoms of childhood ALL are fever (high temperature) and fatigue (excessive tiredness).
Other symptoms may include:
A child with ALL may not show the typical signs of infection but if they show any of the following signs or symptoms you should contact their GP or the hospital straight away:
There are several tests which are carried out to confirm a diagnosis of ALL. Sometimes test results can take a little while. This can be an anxious and worrying time but please remember that it is important that your child’s medical team reach the correct diagnosis so that your child can receive the right treatment.
Tests may include:
Full Blood Count
A blood sample is examined using an automatic cell-counting machine and by examining a stained film under a microscope. The cell-counter will usually indicate that there are large numbers of abnormal white cells (lymphoblasts) in the blood. The appearance of the stained blood cells is usually very typical of ALL. In rare cases, the blood count may be normal, or there may be lower than normal numbers of white blood cells.
Bone marrow examination
If the results of a blood sample show that your child may have ALL, or another serious blood condition, a bone marrow sample will be taken, usually from the pelvis bone. This is normally done under a general anaesthetic or sedation (older children may prefer to have a local anaesthetic) and does not take very long. The bone marrow sample is important to confirm the diagnosis and also for comparison with later samples to show how your child’s ALL is responding to treatment.
There are a number of special investigations which can be done to confirm the diagnosis and to help in planning treatment.
These tests include cytogenetics, which looks at how the chromosomes in your child’s leukaemia cells are different from in their normal cells, and immunophenotyping, which shows exactly what type of ALL your child has. A test called MRD (minimal residual disease) will be done during treatment. MRD testing can detect even very small numbers of remaining leukaemia cells, which is important in planning the next phase of treatment.
The results of these specialised tests will be used in planning what treatment your child should receive. The cytogenetics test will include testing for an abnormal gene called BCR-ABL. This is seen much more often in adult ALL than in childhood ALL; when it is present, standard treatment does not work very well but a type of drugs called tyrosine kinases can be very effective.
Some of the tests will be repeated from time to time, during your child’s treatment and after this is finished. This is to see how well treatment is working, and to make sure that the ALL has not returned after treatment.
Your child will also have X-rays taken and other types of scans to check for infection and to see what parts of your child’s body are affected by the ALL. ALL cells can sometimes get in to the fluid around the brain and spinal cord (cerebrospinal fluid or CSF). For this reason, doctors will take a sample of this fluid to look for leukaemia cells. This is called a lumbar puncture and, like bone marrow sampling, is usually done under local anaesthetic and sedation in older children, and a general anaesthetic in younger children. Again, this test will need to be repeated from time to time during and after treatment.
At any time, you can ask the doctors, nurses or other healthcare workers about your child’s diagnosis and treatment.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is the main part of treatment for ALL in children. The most common cell killing drugs used to treat ALL are:
It has also been found that steroids are very effective in the treatment of ALL. The steroids are artificial versions of natural substances found in the body. The steroid normally used for treatment of ALL is called dexamethasone.
Other drugs will be given to prevent infections and manage side effects of ALL treatments. Some side effects can be unpleasant, ranging from hair loss (which normally grows back quickly) to infertility (which, depending on your child’s age, may be dealt with by storing sperm of eggs).
In order to make treatment easier, most children with ALL will have a central line inserted. This is a long, thin tube which goes into a blood vessel in your child’s chest. If your child has a central line, you will be shown how to look after this, to stop it from becoming blocked or from getting infected.
UKALL 2011 Clinical Trial
UKALL 2011 is designed to test whether some children can safely have a lower total of treatment amount (schedule A), and whether other child may benefit from having more intensive treatment (schedule C). Most children will receive standard treatment (schedule B). UKALL 2011 includes several treatment options, picked at random, to compare the results of slightly different treatment options.
You will be given full information, and a chance to ask questions, before deciding whether or not you would like your child to take part. If you decided that your child will not take part, he or she will receive standard ALL treatment.
Stages of treatment
The exact length of each stage of treatment, and the choice and doses of drugs used at each stage will vary according to the results of the tests, and whether your child is in the trial. You will be given detailed information about your child’s treatment and can ask questions at any time.
The aim of the initial treatment is to reduce the number of abnormal cells to as low as possible (ideally to complete remission). This uses a combination of drugs given over four weeks, and usually the child will be in hospital the whole time. At the end of induction treatment, an MRD test will be done and the results of this will be used to plan the next phase of treatment.
The second phase of treatment is intended to destroy any remaining leukaemia cells. It uses fewer drugs and lower doses than the induction treatment and usually take 8-12 weeks. The exact type of consolidation will depend on the results of the MRD test.
Interim maintenance phase
This is a period of about two months of less intensive treatment before the forth phase. During this period, all of your child’s treatment will be oral (tablets) and they will be at home, unless they have an infection or become poorly and need to go into hospital.
The fourth stage of treatment lasts for seven or eight weeks. The aim of this stage is to ‘mop-up’ any remaining leukaemia cells. Although this uses stronger treatment than interim maintenance, your child can normally be at home unless they develop any problems.
The last stage of treatment is the longest (two years for girls, three years for boys) and is the gentlest. This is needed to prevent the disease from coming back. It involves oral treatment with Methotrexate (weekly) and 6-mercaptopurine (daily) along with Vincristine IV chemotherapy every three months and oral steroids along with the Vincristine for 5 days.
During this stage, blood counts will be followed up regularly and intrathecal methotrexate will be given by lumbar puncture every three months.
Central Nervous System (CNS) treatment
In children with ALL, it is possible for leukaemia cells to get into the fluid around the brain and spine (cerebrospinal fluid or CSF). This is called central nervous system disease and, because drugs in the normal way cannot penetrate into the CSF, it is necessary to give additional treatment to deal with this. This is normally given during the consolidation stage and may consist of radiation treatment to the head and spine, injection of drugs into the CSF by lumbar puncture or high doses of anti-leukaemia drugs. Some types are more likely than others to involve the CNS but the majority of the treatment in the CSF is given for disease prevention.
In boys, the testicles are another place in which leukaemia cells can escape the effects of treatment. For this reason, it may be necessary for boys with ALL to have radiotherapy to the testicle.
Stem cell transplantation
This is not often used in childhood ALL because of the good results of standard treatment. This means having strong chemotherapy, along with radiation therapy, to kill off the bone marrow followed by transplant of healthy stem cells from a matched donor.
Although treatments do come with some side effects, your child is unlikely to experience all of them. Short term side effects can last for a few days or weeks, but for some, can last for the duration of treatment.
Short term side effects can include:
The fatigue will improve when treatment ends, but it can take 6-12 months following treatment until your child will feel back to normal.
Loss of fertility
Some of the drugs used can cause temporary and permanent infertility. Your child’s doctor will talk to you about this in more detail before treatment starts.
If your child is having treatment for ALL, you should discuss the options for protecting your child’s fertility with their doctor. Some drugs have less effect on fertility than others, and it is often possible for a child successfully treated for ALL to later have healthy babies. Unfortunately, children who’ve had stem cell transplant after high doses of chemotherapy or whole body irradiation are more likely to be permanently infertile.
Evidence from clinical studies has shown that any cancer treatment a child has doesn’t lead to an increased risk of cancer or other health problems in their children.
Anthracyclines, drugs used to treat ALL, may affect the heart. This doesn’t affect everyone and if it does occur, it’s usually a temporary side effect because healthcare teams are careful to limit the doses your child has. However, in some people, it can lead to long term heart problems. Your child’s heart function will be carefully monitored during and after treatment, and the drugs given may be altered if any heart problems occur.
Almost all children have a good initial response to treatment with blood counts returning to normal, which is called remission. Unfortunately, in some cases the disease will come back, this is known as relapse. When a relapse happens while a child is on maintenance treatment, there is a good chance that this will respond well the repeating the initial treatment.
When relapse happens early in treatment, this tends not to respond to repeating the previous treatment. In this case, one treatment option is a stem cell transplant. If your child has a relapse or is being considered for a stem cell transplant, you will be given detailed information and a chance to ask questions about what this means.
This type of care includes treatment to prevent infections and to manage them when they happen as well as treatment to deal with side effects.
Because both ALL and its treatment affect the body’s ability to produce healthy blood cells, most children with ALL need transfusions of red blood cells and often of platelets. If this is the case, your child will receive injections of growth factors, which help the body to produce more white cells. This will reduce the frequency and severity of infections.
Much of the supportive care is based on good nursing care, but protecting your child from infection outside of the hospital is also very important. If your child is at school or a playgroup, then measles and chickenpox are particular risks.
It is very important that any child who has been treated for ALL should have a follow-up programme in place to watch for late effects. Once treatment is finished, your child will have regular check-ups at the hospital. These will be frequent at first, probably one to two months, then every few months until they become yearly at five years and onwards.
The purpose of follow-up is to monitor your child and look for signs of relapse or complications and to monitor their growth.
Most of the improvements in the outcome of childhood ALL have come from improvements in supportive care and in finding better ways to use existing drugs. One important area of research is into ways to use the patient’s immune system to kill off leukaemia cells. If successful, this approach would cause much less damage to healthy tissues and less severe long-term effects of treatment.
Much of the research on potential new drugs is being carried on in adults with ALL, who have poorer results with standard treatment. If new drugs are developed, it is likely that there will be clinical trials to see if they may be a good option for treatment of children.