Acute Myeloid Leukaemia

© Channel 4/Hollyoaks

Bad news about your child's health is never easy to take, but when Nancy (Hollyoaks) is told that baby Charlie has Acute Myeloid Leukaemia and needs an urgent bone marrow transplant, her world is turned upside down. But how dangerous is this form of leukaemia, and what are Charlie's chances of survival?

what is acute myeloid leukaemia?

'Acute' describes a condition, which progresses rapidly and has severe consequences.

'Myeloid' means relating to your bone marrow or spinal cord.

'Leukaemia' describes a group of diseases, which cause your bone marrow or blood-producing organs to produce too many of a certain type of white blood cell. The excess production then suppresses your body's production of normal white blood cells, red blood cells and platelets.

Acute myeloid leukaemia (AML) therefore is a rapidly progressing leukaemia, which causes your bone marrow to produce too many of a certain type of white blood cell (or 'blast cell').

Specifically with AML, these cells fail to repair and reproduce themselves in a controlled way, but continue to divide without maturing correctly. These immature cells fail to function properly, while also filling up the bone marrow preventing it from manufacturing healthy blood cells. As a consequence of both of these problems, the body becomes extremely prone to infection.

how common is it?

AML affects approximately 2,000 adults and 50 children per year in the UK. Although it's a relatively rare disease, accounting for approximately 1.2% of cancer deaths in the UK, its incidence is expected to increase as the population ages.

what is the prognosis, or expected outcome, after diagnosis?

In general terms the chances of initial remission are between 50 and 80%. The longer term chance of survival depends on the risk factor (determined by chromosome analysis). For the 'good risk' category there is around 60% or better chance of being alive and well after five years. In other categories of 'standard risk' and 'poor risk', it is hard to predict the outcome.

what causes it?

The exact causes are unknown, and in most cases it is unclear why leukaemia has developed. Research into possible causes is going on all the time.

• Radiation: People exposed to high levels of radiation, such as nuclear industry accidents, have a higher risk of developing leukaemia.



• Smoking: It is thought that this may be due to the concentrated levels of benzene in cigarette smoke. In very rare cases, AML may occur after long-term exposure to benzene (and possibly other solvents) used in industry.



• Anti-cancer treatments: Rarely chemotherapy or radiotherapy can cause leukaemia to develop some years later, known as secondary leukaemia or treatment-related leukaemia. The risk is increased when certain types of chemotherapy drugs are combined with radiotherapy.



• Blood disorders: People with myelodysplasia or some genetic disorders, including Down's syndrome, have a higher risk of developing AML. It is not caused by an inherited faulty gene.

Note: AML isn't infectious and can't be passed on to other people.

what are the symptoms?

The main ones include:

• Looking pale, feeling tired and breathless, which is due to anaemia caused by a lack of red blood cells



• Having more infections than usual, because of a lack of white blood cells



• Unusual bleeding, caused by too few platelets. This may include bruising easily without any obvious cause, bleeding gums, frequent nosebleeds, and heavy periods in women. Some people have a rash of tiny, flat red spots on the skin of the legs or in the mouth, called petechiae.



• Feeling generally unwell and run down



• Having a fever and sweats. This may be due to an infection or to the leukaemia itself.

Other, less common, symptoms may be caused by a build up of leukaemia cells in a particular area of the body, such as:

• Aching bones, caused by pressure from a build up of immature cells in the bone marrow



• Raised bluish-purple areas under the skin – due to leukaemia cells in the skin



• Swollen gums, caused by leukaemia cells in the gums



• Occasionally, a person has no symptoms and the leukaemia is discovered during a routine blood test.

The symptoms of AML may appear over a few weeks, and people often feel ill quite quickly. Treatment needs to be given as soon as possible. If you have any of the above symptoms you should have them checked by your doctor straight away – but remember, they are common to many illnesses other than leukaemia.

how is it diagnosed?

Your GP will examine you and take a blood test – if the results are abnormal, he or she will refer you to hospital for advice and treatment from a haematologist, a doctor who specialises in the treatment of blood problems. The haematologist will ask you questions about your health and about any previous illnesses you have had and then examine you to find out how you are physically. You will have a blood sample taken to check the numbers of all the different types of blood cell in your blood (a full blood count). If the blood test shows that leukaemia cells are present, the haematologist will want to take a sample of your bone marrow to plan the best treatment for you.

how is a bone marrow sample taken?

It's usually taken from the back of your hipbone (pelvis) under a local anaesthetic. You will be given a small injection to numb the area and the doctor will gently pass a needle through the skin into the bone and draw a small sample of liquid marrow into a syringe to be looked at later under the microscope (bone marrow aspirate). He or she will then take a small core of marrow from the bone (a trephine biopsy). The sample is looked at under a microscope by a pathologist, who will identify the type of leukaemia. They will also count the number of immature blood cells (blasts) in the sample. Other tests will also be carried out on the bone marrow sample to help confirm the diagnosis.

how is it treated?

The aim of treatment for AML is to destroy the leukaemia cells and allow the bone marrow to work normally again. When there is no sign of the leukaemia and the marrow is working normally this is called 'remission'. For many people with AML the remission lasts indefinitely and the person is said to be cured. Chemotherapy drugs are the main treatment used and have been shown to be very effective in treating AML. Most people with AML go into remission after chemotherapy, and many people are cured.

In some situations high-dose chemotherapy and a stem cell or bone marrow transplant are used to improve the chances of curing the leukaemia.

People who have a type of AML called acute promyelocytic leukaemia (APL) are usually treated with a drug called ATRA (All Trans-Retinoic Acid). It is a specialised form of vitamin A and is also known as tretinoin (Vesanoid®).

ATRA is given for up to three months alongside chemotherapy treatment. It makes the leukaemia cells mature (differentiate), and so can reduce leukaemia symptoms very quickly. Your doctor will plan your treatment by taking into account a number of factors, including your age, general health, and the type of abnormal genes that are present in the leukaemia cells.

how do transplants work?

Bone marrow and stem cell transplants are a way of allowing much higher doses of chemotherapy to be given, to improve the chances of completely curing the leukaemia. Bone marrow transplantation is usually only used for children with AML that is likely to come back following standard chemotherapy, or for children whose leukaemia has come back (recurred) following standard treatment. They are not suitable treatments for everyone with ACM.

Very high doses of chemotherapy, with or without radiotherapy, are given, which destroy all the cells in your bone marrow. Soon afterwards, stem cells (the immature blood cells which develop into red cells, white cells and platelets) are given back into your blood through your central or PICC line. These cells make their way into the bone marrow and start to make healthy blood cells.

The stem cells may be your own (taken before your high dose treatment), or donated by someone else (usually a brother or sister). The new stem cells take a few weeks to settle in your bone marrow and produce the blood cells that you need. During this time you will have very low immunity. You will probably need to stay in hospital and be nursed in isolation. This is done to reduce your risk from infection until your white blood cell count has recovered.

Stem cell transplants use stem cells which are taken from the blood. This is collected through a small tube (cannula) which is put into a vein. The stem cells are then removed, and the blood is then returned through another cannula.

For a bone marrow transplant, some of the bone marrow is taken from the bones under general anaesthetic. The bone marrow also contains stem cells.

Stem cell and bone marrow transplants carry some risk, and are generally carried out in major cancer treatment hospitals, so you may have to be treated at a hospital some distance from your home.

how can I donate my bone marrow?

Sometimes an appropriate donor can be found within the patient's immediate family as it is more likely that their 'tissue type' will match. However, only 30% of donors are found this way, which is why many leukaemia patients rely on the British Bone Marrow Registry to find another suitable donor.

It is your genes that determine your tissue type, but there are other factors that determine the probability of finding a compatible tissue typed donor for a patient. Some tissue types are more common in particular ethnic backgrounds. Therefore, in addition to matching a donor and patient's tissue type it's also important to match the ethnic background. By doing this, the transplant has a better chance of being successful. Bone marrow donors are always needed, so volunteering yourself could well save someone's life. The primary criteria are that you have to be between 18 and 49 and either are prepared to or have given blood. Go to: www.blood.co.uk/pages/marrow_info.html or call 0845 7 711 711 for more information and details of where you can go to register.

help and info

Channel 4 is not responsible for the content of third party sites.

organisations

Leukaemia Care
One Birch Court
Blackpole East
Worcester WR3 8SG
Helpline: 0800 169 6680 free24-hour helpline
Email: enquiries@leukaemiacare.org.uk
Website: www.leukaemia care.org.uk
Aims to provide care and support for everyone affected by leukaemia including families and carers.

Leukaemia Research
43 Great Ormond Street
London WC1N 3JJ
Helpline: 020 7269 9060 (Mon-Fri 9am-5pm)
Email: info@lrf.org.uk
Website: www.lrf.org.uk
Dedicated to researching blood cancers and disorders including leukaemia, Hodgkin;s and other lymphomas, and myeloma.

Anthony Nolan Trust
Units 2-3 Heathgate Place
75-87 Agincourt Road
London NW3 2NU
Tel: 020 7284 1234
E-mail: healthgate@anthonynolan.com
Website: www.anthonynolan.org.uk
The Trust recruits and keeps a register of potential bone marrow donors.

websites

Cancerbackup
Helpline: 0808 800 1234 free helpline
www.cancerbackup.org.uk
Has detailed information about AML including treatment by chemotherapy and bone marrow transplant.

African Caribbean Leukaemia Trust
www.aclt.org
It aims to raise the awareness of the need for people of African descent to become bone marrow donors.

Cancer Research UK
Tel: 0800 226 237 (9-5 Mon-Fri)
www.cancerhelp.org.uk
You can phone cancer information nurses or email them. Their website provides a detailed section about acute leukaemia including its diagnosis and treatment.

Patient UK
www.patient.co.uk/showdoc/27000843
Useful information about AML including causes, symptoms and treatment.

(January 2008)