About acute lymphoblastic leukaemia (ALL)
Types of ALL
The World Health Organisation uses a classification system for ALL. These different classifications include:
Pre-B-cell ALL
In between 75-80% of adult cases, ALL arises in B-lymphocytes in the early stages of development in the bone marrow. The disease is therefore called precursor B-cell ALL or Pre-B-cell ALL.
B-cell ALL
B-cell ALL arises in more mature developing lymphocytes. This type of ALL is less common accounting for around 3-5% of all adult cases. B-cell ALL is sometimes called Burkitt-like or Burkitt type ALL. People diagnosed with B-cell ALL are commonly treated with similar drugs to those used to treat Burkitt lymphoma.
T-cell ALL
In around 20-25% of cases, ALL arises in developing T-cells. This type of ALL can be further classified as early, mid or late, depending on the maturity of the affected cell. T-cell ALL commonly presents with a high white blood cell count and involvement of the central nervous system at diagnosis.
How common is ALL?
More than 300 adults and children are diagnosed with ALL each year in Australia.
Who gets ALL?
ALL can occur at any age but is more common in young children (0-14 years) who represent close to 60% of all cases. ALL is the most common type of childhood leukaemia, and the most common childhood cancer. It is more common in males than females.
The characteristics of ALL differ greatly between children and adults. These days with treatment, the majority of children with ALL can be cured of their disease. In adults, cure rates are more variable.
Causes of acute lymphoblastic leukaemia (ALL)
The exact causes of ALL remain largely unknown but it is thought to result from mutations in one or more of the genes that normally control blood cell development. This mutation will result in abnormal growth.
Research is going on all the time into possible causes of this damage, and certain factors have been identified that may put some people at an increased risk. These include exposure to:
- very high doses of radiation either accidentally (nuclear accident) or therapeutically (to treat other cancers)
- industrial chemicals like benzene, pesticides, and certain types of chemotherapy used to treat other cancers
- certain types of viral infections and the way in which the immune system reacts may play a role in the development of some types of ALL
- people with certain genetic disorders like Down’s syndrome and Fanconi’s anaemia may have a higher than average risk of developing ALL.
Symptoms of acute lymphoblastic leukaemia (ALL)
The main symptoms of ALL are caused by a lack of normal circulating blood cells. ALL develops quickly, so people are usually only unwell for only a short period of time (it could be days, or weeks) before they are diagnosed.
Common symptoms of ALL can include:
- anaemia due to a lack of red cells. Anaemia can cause persistent tiredness, dizziness, paleness, or shortness of breath when physically active.
- frequent or repeated infections and slow healing, due to a lack of normal white cells, especially neutrophils
- increased or unexplained bleeding or bruising, due to a very low platelet count
- bone and/or joint pain as a result of the marrow being overcrowded with leukaemic cells.
Other symptoms may include swollen lymph nodes (glands), chest pain and abdominal discomfort due to a swollen spleen or liver.
Occasionally people have no symptoms and ALL is diagnosed during a routine blood test. Some of these symptoms described may also be seen in other illnesses, including viral infections, so it is important to see your doctor so that you can be examined and treated properly.
Diagnosis of acute lymphoblastic leukaemia (ALL)
ALL is diagnosed by examining samples of your blood and bone marrow in a variety of tests.
Full blood count
The first step in the diagnosis is a simple blood test called a full blood count (FBC) or complete blood count (CBC). This involves a sample of blood from a vein in your arm being sent to the laboratory for investigation. Many of the white blood cells may be abnormal leukaemic blast cells and the presence of these blast cells suggests you have ALL. An ALL diagnosis needs to be confirmed by examining the cells in your bone marrow.
Bone marrow examination
If the results of your blood tests suggest that you might have ALL, 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, a clinic, or a 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. The diagnosis of ALL is confirmed by the presence of an excessive number of blast cells in the bone marrow.
Further testing
Once the diagnosis of ALL is made, blood and bone marrow cells are examined further using special laboratory tests. These include immunophenotyping, cytogenetic and molecular tests. These tests provide more information about the exact type of disease you have, the likely course of your disease and the best way to treat it. A small sample of the cerebo-spinal fluid (CSF) that surrounds your brain and spinal cord is also collected, during a procedure called a lumbar puncture. This fluid is tested in the laboratory to check for the presence of leukaemic cells within the central nervous system.
Other tests
Tests may be conducted to provide information on your general health and how your vital organs are functioning. These include a combination of further blood tests and imaging tests (x-rays, scans and ECG). These results will provide a baseline of your disease and general health which will be compared with later results to assess how well you are progressing and responding to treatment.
Treatment of acute lymphoblastic leukaemia (ALL)
Because it progresses quickly, treatment needs to begin soon after ALL is diagnosed. The type of treatment used will depend on a number of factors including the sub-type of ALL, the genetic make-up of the leukaemic cells, your age and your general health.
Chemotherapy
Chemotherapy is the main form of treatment for ALL. A combination of drugs, including steroids, is usually given in several cycles with a rest period of a few weeks in between. Initially, the aim of treatment is to destroy leukaemic cells and induce a remission. This means that there is no evidence of leukaemic cells in the blood and bone marrow and that normal blood cell production and blood counts are restored. In some cases, where there is a high risk that the leukaemia will relapse, patients may be offered even more intensive therapy followed by a stem cell transplant.
Chemotherapy is given in many different ways to treat ALL. This includes intravenously (into a vein), intramuscularly (into a muscle) and in tablet form. To prevent and treat disease in the brain and spinal cord (CNS) chemotherapy is injected intrathecally, directly into the fluid that surrounds these structures. Sometimes, this area is also treated using radiotherapy. In males, radiotherapy may be given to the testes to treat relapsed disease in this area.
Treatment for ALL can be divided into three phases:
- induction therapy
- post-remission (consolidation) therapy
- maintenance therapy.
Induction therapy
Soon after you are diagnosed your doctor will need to begin an intensive course of treatment to bring about, or induce, a remission. You will need to be admitted to hospital for this first phase of treatment. Sometimes the disease does not respond to treatment as expected and you may be said to have resistant or refractory disease. In these cases the doctor may recommend a more intensive form of therapy to treat your disease more effectively.
Post-remission (consolidation) therapy
Soon after induction therapy finishes and remission is achieved, more treatment is required to help destroy any leftover disease in your body. This is important because it helps to prevent the disease from reappearing (relapsing), or spreading to the central nervous system (brain and spinal cord) in the future. The type of consolidation therapy chosen for you will depend on your estimated risk of relapse in the future, in other words the ‘risk group’ to which you belong Consolidation therapy usually involves ‘blocks’ of treatment over several months. Some people may be offered more intensive treatment followed by a stem cell transplant, to more effectively treat their disease.
Maintenance therapy
Maintenance therapy is designed to help keep your disease in remission and prevent it from reappearing (relapsing) in the future. Common maintenance protocols involve chemotherapy tablets — some taken daily and others weekly — and possibly blocks of injections of chemotherapy with courses of cortico-steroids. This phase of treatment usually lasts for several months during which time you will be treated as an outpatient; however sometimes you may need to be admitted to hospital.
Your prognosis is an estimate of the likely course of your disease and whether it is likely to relapse in the future. It provides some guide regarding the chances of curing the disease or controlling the disease for a given time. Your doctor is the best person to give you an accurate prognosis regarding your leukaemia as he or she has all the necessary information to make this assessment.
Side effects of ALL 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 experience any of the following:
- a temperature of 38oC or higher (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, or 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 ALL treatment?
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 you are 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.
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 leukaemia.
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.
Parotitis
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.
Caring for someone with acute lymphoblastic leukaemia (ALL)
We have a range of information and resources that may help when you are caring for someone with acute lymphoblastic leukaemia (ALL).
