Leukemia





What Is Leukemia?

Leukemia is a type of cancer that affects the blood and bone marrow, the spongy center of bones where our blood cells are formed. The disease develops when blood cells produced in the bone marrow grow out of control.

About 43,050 people are expected to develop leukemia in 2010.Common Types of Leukemia

The four most common types of leukemia are:

- acute myeloid leukemia (AML) - cute lymphoblastic leukemia (ALL) - chronic myeloid leukemia (CML) - chronic lymphocytic leukemia (CLL)

Each main type of leukemia is named according to the type of cell that's affected (a myeloid cell or a lymphoid cell) and whether the disease begins in mature or immature cells.

Acute Lymphocytic Leukemia




ALL is also called acute lymphocytic leukemia and acute lymphoid leukemia.

ALL is the most common type of cancer in children from 1 to 7 years old.

ALL is the most common type of leukemia in children from infancy up to age 19.

ALL affects the blood cells and immune system. There are several ALL subtypes.

The type of treatment you receive and your treatment outcome depend on your ALL subtype and individual risk factors.Overall survival statistics for people with ALL are 66.4 percent (all ages) and 90.8 percent for children under 5 years old, according to the National Cancer Institute.

How ALL Develops

ALL results from an acquired genetic injury to the DNA of asingle cell in the marrow. The effects of ALL include uncontrolled and exaggerated growth and accumulation of cells called “lymphoblasts” or “leukemic blasts,” which fail to function as normal blood cells.The presence of the leukemic blasts blocks the production of normal cells. As a result, when ALL is diagnosed, the number of healthy blood cells (red cells, white cells and platelets) is usually lower than normal.

Signs and Symptoms

It is common for a person with ALL to feel a loss ofwell-being because of the underproduction of normal cells in the bone marrow. The person may tire more easily and have shortness of breath during normal physical activities.To begin determining the reason for these signs and symptoms, your doctor will want to examine your blood by doing a blood test called a complete blood count (CBC). Low red cells, white cells and platelets are common in patients with newlydiagnosed ALL.

Other signs and symptoms that a person with ALL may have include

• A pale complexion from anemia

• Signs of bleeding caused by a very low platelet count, including

• Black-and-blue marks or bruises occurring for no reason or because of a minor injury

• The appearance of pinhead-sized red spots on the skin, called “petechiae”

• Prolonged bleeding from minor cuts

• Mild fever

• Frequent minor infections

• Discomfort in bones or joints

• Enlarged spleen, liver or lymph nodes.

Leukemic cells can also collect in the testes in a small number of patients.

Bleeding.

A low platelet count predisposes patients to bleeding. Bleeding in the brain or lung is serious and can be fatal. However, such bleeding is usually preceded by minor bleeding, such as nose bleeds, blood in the urine or bruises.

Infection

Severe infection usually does not occur at the time of diagnosis. If the neutrophil count becomes or remains low because of ALL or its treatment, serious infection almost invariably occurs and is a leading cause of death from ALL.

A person with signs or symptoms that suggest the possibility of leukemia is usually referred to a specialist. This may be a hematologist/oncologist. The specialist willorder additional tests to make a diagnosis (see below). The signs and symptoms of ALL are associated with a number of other, less serious diseases. An accurate diagnosis of the type of leukemia is important. The exact diagnosis helps the doctor to

• Estimate how the disease will progress

• Determine the appropriate treatment.

Talk to your doctor about The diagnostic tests that are being done: What the results mean; Getting copies of the test results.

Blood and Bone Marrow Tests

Blood and bone marrow cells are examined to diagnose ALL and identify the ALL subtype. An examination of the stained (dyed) blood cells with a light microscope will often show the presence of leukemic blast cells (immature cells that do not functionlike normal, mature white blood cells). A bone marrow examination is preferred to diagnose ALL because a proportion of patients do not have leukemic blasts circulating in the blood at the time of diagnosis.

Who gets it?

ALL can occur at any age but is more common in young children (0-14 years) who represent close to 60 per cent 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 in females.

What causes 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 . 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 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.

What are the symptoms?

The main symptoms of ALL are caused by a lack of normal blood cells. These include:

- anaemia due to a lack of red cells ; causing persistent tiredness, dizziness, paleness, or shortness of breath. - frequent or repeated infections and slow healing, due to a lack of normal white blood cells, especially neutrophils.

- increased or unexplained bleeding or bruising, due to a very low platelet count.

Other symptoms may include bone pain, swollen lymph nodes, chest pain and abdominal discomfort due to a swollen spleen or liver.

How is it diagnosed?

ALL is diagnosed by a full blood count (FBC) and a bone marrow biopsy/examination.

How is it treated?

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 general health.

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. Once a remission has been achieved, further treatment is needed to help destroy any left over disease, and try to prevent leukaemia from coming back (relapsing) in the future.

Chemotherapy is given in many different ways to treat ALL. This includes intravenously (into a vein), intramuscularly (into a muscle) and in tablet form.

Leukaemic cells can collect in the brain and spinal cord (central nervous system). To prevent and treat disease in this area, 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. This can cause infertility.

Treatment is generally 'risk-based'. This means that you will assigned to a particular treatment plan (protocol) depending on a number of factors, most importantly your risk of relapse in the future. Treatment can last from two to three years or longer depending on the treatment protocol being followed and how well you are progressing and responding to treatment.

People with a particular genetic abnormality found in their leukaemic cells, called the Philadelphia (Ph) chromosome, are also given a drug called imatinib mesylate (Glivec®). This works by blocking the leukaemia-causing effects of the abnormal enzyme tyrosine kinase, which forces leukaemic cells to die.

Occasionally, a stem cell transplant may be used to treat relapsed ALL, or where there is a high likelihood that ALL will relapse in the future.

Many children who are treated for ALL can be cured of their disease. The cure rates for adults with ALL is more variable.What are the side effects of treatment?

All treatments can cause side effects. The type and severity however will vary between individuals, depending on the type of treatment used and how an individual responds to it. 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.

In general, more intensive treatments like stem cell transplant, and treatments that involved radiation to the central nervous system, or total body irradiation (TBI) are associated with more severe side-effects. They can also cause more significant long-term effects, especially in children.

Although ALL affects the bone marrow's ability to produce adequate numbers of blood cells and platelets, chemotherapy affects this ability further. Blood counts generally fall within a week of treatment and may take some time to recover, depending on the type and doses of drugs used. During this time you are likely to need antibiotics and other drugs to treat, or prevent infection. You are also likely to need blood transfusions to treat severe anaemia, and platelet transfusions to reduce the risk of bleeding.

Other possible side effects of chemotherapy include:

- feeling sick - nausea and/or vomiting - feeling tired and weak - hair loss and thinning - mouth problems such as mucositis or ulcers

- diarrhoea or constipation - skin problems such as dryness, rash or sensitivity to sunlight fertility problems

Side-effects of steroids depend largely on how long they are used for, and the dose given. Short-term use may cause increased appetite, restlessness or difficulty sleeping. Longer-term use may lead to fluid retention, raised blood sugars and increased susceptibility to infections.

Your doctor and nurse will discuss with you the possible side-effects of your treatment and how they can be managed.




Chronic Lymphocytic Leukemia



What is Chronic Lymphocytic leukaemia (CLL)?

Chronic lymphocytic leukaemia (CLL) is a type of slow growing leukaemia that affects developing B-lymphocytes. B lymphocytes (also known as B-cells) are specialised white blood cells. Under normal conditions they produce immunoglobulins (also called antibodies) that help protect our bodies against infection and disease. In people with CLL, lymphocytes undergo a malignant (cancerous) change and become leukaemic cells.

It is important to emphasise here that for many people CLL remains stable for many months and years and has little if any impact on their lifestyle or general health. Around 30 percent of people diagnosed with CLL never require any treatment for their disease and can survive for many years despite their diagnosis. For others, the leukaemic cells multiply in an uncontrolled way. Live longer than they are supposed to and accumulate in the bone marrow, blood stream, lymph nodes (glands), spleen, liver and other parts of the body. These cells are abnormal and as such they are unable to function properly. Over time, an excess number of lymphocytes crowd the bone marrow, and interfere with normal blood cell production. The bone marrow produces inadequate numbers of red cells, normal white blood cells and platelets, making some people with CLL more susceptible to anaemia, recurrent infections and bruising and bleeding easily. Circulating red blood cells and platelets can also be damaged by abnormal proteins made by the leukaemic cells.

CLL usually develops slowly and progresses slowly, over months and years. Most people have no symptoms of their disease when they are first diagnosed. In these cases, people often require no treatment for a long time, apart from regular checkups with their doctor to carefully monitor their health. Others may need to be treated soon after they are diagnosed.How common is it?

Each year in Australia around 718 people are diagnosed with CLL*, making it the most common type of leukaemia. It is a rare disease overall however, accounting for around 0.8 per cent of all cancers diagnosed.Who gets it?

The risk of developing CLL increases with age. Almost 80 per cent of all new cases are diagnosed in people over the age of 60 years. CLL is rare in people under 40. It occurs more frequently in men than in women.What causes CLL?

The causes of CLL remain unknown but it is thought to result from damage to one or more of the genes that normally controls blood cell development . A family history may put some people at higher risk of developing CLL.What are the symptoms?

Because CLL develops slowly many people don't have any symptoms, particularly in the early stages and the disease is picked up during a routine blood test.

For others, possible symptoms may include:

- swollen lymph nodes (glands) in the neck, under the arms or in the groin, due to collections of lymphocytes in these areas - pain or discomfort under the ribs on the left side, due to an enlarged spleen - anaemia, due to a lack of red cells ; causing 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 blood cells

- increased or unexplained bleeding or bruising, due to a very low platelet count

- excessive sweating at night - unintentional weight loss

How is it diagnosed?

CLL is diagnosed by a full blood count (FBC) and a bone marrow biopsy/examination.

How is it treated?

CLL is generally a slow-growing disease and many people, particularly in the early stages, do not need treatment. Instead the doctor may recommend regular check-ups to carefully monitor their health. Treatment usually only starts once the disease begins to progress, or cause troublesome symptoms. Many people with CLL have the disease for years without it causing any problems.

Treatment can involve chemotherapy, which may be given either in tablet form or intravenously, through a vein in your hand or arm. Steroids may also be given. Drugs called monoclonal antibodies , for example rituximab (Mabthera®), may be given along with chemotherapy. This drug works by deliberately targeting abnormal lymphocytes, allowing chemotherapy to be delivered directly to these cells without causing harmful side effects to other parts of the body.

Occassionally, a stem cell transplant may be offered to some younger patients.

A range of supportive therapies are available to treat symptoms of CLL. These include antibiotics to prevent and treat infections, and blood & platelet transfusions to restore levels of red cells and platelets.

Your doctor and nurse will discuss with you the possible side-effects of any treatments you need and how they can be managed.




Chronic Myeloid Leukemia

What is it?

Chronic myeloid leukaemia (CML) is a type cancer that affects the blood and bone marrow. In CML the bone marrow produces too many white cells, called granulocytes. These cells, sometimes called blasts or leukaemic blasts gradually crowd the bone marrow, interfering with normal blood cell production. They also spill out of the bone marrow and circulate around the body in the bloodstream. Because they are not fully mature, they are unable to work properly to fight infections. Over time, a shortage of red cells and platelets can cause anaemia, bleeding and/or bruising.

CML usually develops gradually, during the early stages of disease, and progresses slowly over weeks or months. It has three phases: the chronic phase , the accelerated phase and the blast phase . These phases are distinguished by the number of blast cells (immature white cells) in the blood and bone marrow, and the severity of symptoms.How common is it?

Each year in Australia around 249 people are diagnosed with CML*. Overall, CML is a rare disease, accounting for around 0.3 per cent of all cancers diagnosed.

Who gets it?

CML can occur at any age but it is more common in adults over the age of 50, who account for nearly 70 per cent of all cases. CML occurs more frequently in men than in women. It is rare in children (0-14 years) with around 4 cases per year diagnosed in this age group.

What causes CML?

Most people diagnosed with CML have a genetic abnormality in their blood cells called the Philadelphia (Ph) chromosome. The Ph-chromosome causes the production of an enzyme called tyrosine kinase which leads to CML. Why this genetic abnormality occurs in the first place remains unknown but there are likely to be a number of factors involved. In a very small number of cases it may result from exposure to very high doses of radiation, either accidentally (nuclear accident) or therapeutically (to treat other cancers).

What are the symptoms?

Because CML develops slowly many people don't have any symptoms, particularly in the early stages and the disease is picked up during a routine blood test.

As the disease progresses, symptoms arise from the increasing number of abnormal blood cells in the bone marrow and blood, and the decreasing number of normal blood cells. Possible symptoms may include:

- anaemia, due to a lack of red cells ; causing persistent tiredness, dizziness, paleness, or shortness of breath when physically active

- increased or unexplained bleeding or bruising, due to a very low platelet count - frequent or repeated infections and slow healing, due to a lack of normal white blood cells - pain or discomfort under the ribs on the left side, due to an enlarged spleen - excessive sweating or unintentional weight loss

How is it diagnosed?

CML is diagnosed by a full blood count (FBC) and a bone marrow biopsy/examination.

How is it treated?

Treatment will vary depending on the phase of disease, your age and general health. During the chronic phase, treatment is used to control CML and keep your blood counts within a normal range. This can involve chemotherapy, which is usually taken in tablet form, at home. A drug called imatinib mesylate (Glivec®), which is given in capsule form, is also used. Glivec® works by blocking the leukaemia-causing effects of an abnormal enzyme called tyrosine kinase, forcing the leukaemic cells to die. A stem cell transplant may be an option for some younger patients, providing them with a better chance of cure.

In the accelerated phase, the disease starts to progress more quickly. Blood counts become more abnormal and people start to develop symptoms of their disease. In the blast or blast crisis phase blood counts become very abnormal and the numbers of blast cells in the blood and bone marrow increase dramatically. The treatment of accelerated and blast phase CML usually involves a more intensive approach, involving a combination of chemotherapy drugs given intravenously (into a vein). You need to be admitted to hospital for this type of treatment. The aim of treatment during the advanced stages of CML is to destroy the leukaemic cells and allow the bone marrow to function normally again, or to return the patient to the chronic phase of their disease.

Some people are diagnosed with an extremely high number of white cells in their blood. A process known as leukopheresis may be needed to remove these cells, which could otherwise cause problems in the body by clogging up small blood vessels. This process is similar to dialysis where all your blood is passed through a special machine called a cell separator. This machine separates and collects the excess white cells and returns the rest of your blood to you. Chemotherapy is also used to reduce a high white cell count at diagnosis.

What are the side effects of treatment?

All treatments can cause side effects. The type and severity however 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.

Your doctor and nurse will discuss with you the possible side-effects of your treatment and how they can be managed.

Acute Myeloid Leukemia



What is it ?

Acute myeloid leukaemia (AML) is a type of cancer that affects the blood and bone marrow. AML is characterised by an overproduction of immature white blood cells, called myeloblasts or leukaemic blasts. These cells crowd the bone marrow, preventing it from making normal blood cells. They can also spill out into the blood stream and circulate around the body. Due to their immaturity they are unable to function properly to prevent or fight infection. Inadequate numbers of red cells and platelets being made by the marrow cause anaemia, and easy bleeding and/or bruising.

Acute myeloid leukaemia is sometimes called acute myelocytic, myelogenous or granulocytic leukaemia.Which type of AML do I have ?

AML is not a single disease. It is the name given to a group of leukaemias that develop in the myeloid cell line in the bone marrow. Some years ago doctors from America, France and Britain decided to classify AML into eight different subtypes based on the appearance of the leukaemic cells under the microscope. Each subtype provides information on the type of blood cell involved and the point at which it stopped maturing properly in the bone marrow. This is known as the French-American-British (FAB) classification system.

The current World Health Organization’s classification system for AML uses additional information, obtained from more specialised laboratory techniques, like genetic studies, to classify AML more precisely. This information also provides more reliable information regarding the likely course (prognosis), of a particular subtype of AML, and the best way to treat it.

The most important factor in predicting prognosis in AML is the genetic make-up of the leukaemic cells. Certain cytogenetic changes are associated with a more favourable prognosis than others. This means that they are more likely to respond well to treatment, and may even be cured. Favourable cytogenetic changes include: a translocation between chromosome 8 and 21 t(8;21), inversion of chromosome 16; inv(16) and a translocation between chromosome 15 and 17; t(15;17). This final change is found in a subtype of AML called acute promyelocytic leukaemia (APML or M3). APML is treated differently to other types of AML, and usually has the best overall prognosis.

Other cytogenetic changes are associated with an average or intermediate prognosis, while others still are associated with a poor, or unfavourable prognosis. It is important to note that in most cases of AML, neither ‘good’ or ‘bad-risk’ cytogenetic changes are found. People with ‘normal’ cytogenetics are also regarded as having an average prognosis.

Some subtypes of AML are associated with specific symptoms. For example, in some subtypes of AML, leukaemic cells can spread from the blood stream into other parts of the body like the gums, causing swelling and discomfort in this area. Acute promyelocytic leukaemia (APML or M3) is associated with bleeding and abnormalities in blood clotting.How common is it ?

Each year in Australia around 715 people are diagnosed with AML*. Overall AML is rare disease, accounting for 0.8 per cent of all cancers diagnosed, at a rate of 3.7 per 100,000 of the population.

Who gets it ?

AML can occur at any age but is more common in adults over the age of 60 years. Around 50 children (0-14 years) are diagnosed with AML in Australia each year. It occurs more frequently in males than in females.

What causes AML?

In most cases the causes of AML remain largely unknown but it is thought to result from damage to one or more of the genes that normally control blood cell development . 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 over a long period, certain types of chemotherapy to treat other cancers and - cancer-causing substances in tobacco smoke

Some people with pre-existing blood disorders like certain myelodysplastic syndromes (MDS) and myeloproliferative disorders (MPD), or certain genetic disorders like Down's syndrome, Bloom syndrome and Fanconi's anaemia may have a higher than average risk of developing AML.

What are the symptoms?

The main symptoms of AML are caused by a lack of normal blood cells. These include:

- anaemia due to a lack of red cells ; causing 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.

Other symptoms may include bone pain, swollen lymph nodes, swollen gums, chest pain and abdominal discomfort due to a swollen spleen or liver.

How is it diagnosed?

AML is diagnosed by a full blood count (FBC) and a bone marrow bipsoy/examination.

How is it treated?

Because it progresses quickly, treatment needs to begin soon after AML is diagnosed. The type of treatment used will depend on a number of factors including the sub-type of AML, the genetic make-up of the leukaemic cells, your age and general health.

Chemotherapy is the main form of treatment for AML. 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 normal blood counts are restored. Once a remission has been achieved, more chemotherapy is given to try to prevent the leukaemia from returning (relapsing). This is called post-remission or consolidation therapy.

Chemotherapy is usually given as a combination of drugs, usually over a period of a week or so. In most cases the drugs are given as infusions through a special line called a central venous catheter, which will be inserted before you start treatment.

People with a sub-type of AML called acute promyelocytic leukaemia (APML) may also be treated with a non-chemotherapy drug called all-trans retinoic acid (ATRA), a derivative of vitamin A which helps make the leukaemic cells either mature properly, or die.

Occasionally, a stem cell transplant may be used which increases the chance of cure for some people with AML.

What are the side effects of treatment?

All treatments can cause side effects. The type and severity however 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.

Although AML affects the bone marrow's ability to produce adequate numbers of blood cells and platelets, chemotherapy affects this ability further. Blood counts generally fall within a week of treatment and may take some time to recover, depending on the type and doses of drugs used. During this time you are likely to need antibiotics and other drugs to treat, or prevent infection. You are also likely to need blood transfusions to treat severe anaemia, and platelet transfusions to reduce the risk of bleeding.

Other possible side effects of chemotherapy include:

- feeling sick - nausea and/or vomiting - feeling tired and weak - hair loss and thinning - mouth problems such as mucositis or ulcers - diarrhoea or constipation - skin problems sych as dryness, ras or sensitivity to sunlight - fertility problems

Your doctor and nurse will discuss with you the possible side-effects of your treatment and how they can be managed.




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