The risk of ALL is lowest between the ages of 25 and 50 and then begins to pick up. African Americans are much less likely to have ALL.

What Causes Acute Lymphocytic Leukemia (ALL) ?
 
The cause of most cases of ALL remains unknown at this time. But some cases can be linked to certain risk factors. At this time, there are no known lifestyle risk factors for ALL.

Risk Factors for ALL:

• Being a survivor of an atomic bomb blast or nuclear reactor accident has been linked to ALL.
• There is some concern about very high-voltage power lines as a risk factor for leukemia. The United States National Cancer Institute (NCI) has several large studies going on now to look into this question. So far, the studies show either no increased risk or a very slightly increased risk. Clearly, most cases of leukemia are not related to power lines.
• A small number of people are at greater risk of acute leukemia because they have certain rare diseases or because the have a certain virus (HTLV-1).

Can Acute Lymphocytic Leukemia Be Prevented?

Most people who develop ALL do not have any of the above risk factors. The cause of their leukemia remains unknown at this time. Because the cause is not known, there is no way to prevent most cases of ALL
 
How Is Acute Lymphocytic Leukemia Found?
 
At this time, there are no special tests that can find acute leukemia early. The best course of action is to report any symptoms to the doctor right away.

Leukemia can cause many signs and symptoms, some of them very general in nature. Keep in mind that the symptoms mentioned below are most often caused by something other than cancer.

General symptoms of ALL can include:

• weight loss
• fever
• loss of appetite
• anemia
• infection
• bruising
• bleeding
• frequent or severe nosebleeds
• bleeding from the gums

Most symptoms of ALL are caused by a shortage of normal blood cells, a result of the crowding out of normal blood cell-producing bone marrow by leukemia cells. As a result, the person doesn't have enough properly working red blood cells, white blood cells, and platelets.

Anemia is a result of a shortage of red blood cells. Anemia causes shortness of breath, fatigue, and a pale skin color.

Not having enough normal white blood cells can increase the risk of infection. Although people with leukemia may have very high white blood cell counts, the cells are not normal and do not protect against infection very well.

Not having enough blood platelets can lead to bruising, bleeding, frequent or severe nosebleeds, and bleeding from the gums.

ALL can spread outside of the bone marrow to other organs . If it spreads to the brain or spinal cord (central nervous system), it can cause symptoms such as:

• headaches
•  weakness
• seizures
• vomiting
• trouble keeping one's balance
• blurred vision
• bone pain or joint pain
• swelling of the liver and spleen

Some people have bone pain or joint pain caused by the spread of cancer cells to the surface of the bone or into the joint.

Leukemia can also cause swelling of the liver and spleen. If the disease has spread to the lymph nodes, these nodes may be swollen.

The T-cell type of ALL often involves the thymus , a gland found in the center of the chest, near the heart. An enlarged thymus can press on the nearby windpipe, causing coughing, shortness of breath, or even suffocation. A large vein, the superior vena cava (SVC) that carries blood from the head and arms back to the heart, also passes next to the thymus. If leukemia cells compress the SVC, swelling of the head and arms (SVC syndrome) can result. This can affect the brain and is life threatening. People with SVC syndrome need treatment right away.

What Testing will be Done If Acute Lymphocytic Leukemia Is Suspected ?
 
If there is reason to think that you might have leukemia, the doctor will need to take samples of cells from your blood and bone marrow to find out if the disease is really present.

Bone marrow tests: In bone marrow aspiration, a thin needle is used to draw up a small amount of liquid bone marrow. During a bone marrow biopsy, a small cylinder of bone and marrow (about ½ inch long) is removed with a slightly larger needle.

Both samples are usually taken at the same time from the back of the hipbone.

• The patient lies face down and the area is cleaned with a special soap
• Before the sample is taken, the doctor injects an anesthetic into area near the back of the hipbone to numb it.
• Then the doctor makes a small cut in order to insert a needle.
• The needle is moved through the bone with a twisting motion.
• Sometimes the needle going into the bone is painful, but it only lasts a short time.
• During aspiration, the sucking out is often painful for a brief moment.

These tests are used to tell whether leukemia is present and also, if you are having treatment, how well the disease is responding.

Excisional lymph node biopsy: In this procedure, an entire lymph node is removed. If the node is near the skin's surface, a simple operation can be done by numbing just the area around the node. But if the node is inside the chest or abdomen, the patient will need general anesthesia (the patient is asleep). This test is only rarely needed for people with leukemia.

Spinal tap (lumbar puncture): In this procedure, a small needle is placed into the spinal cavity in the lower back to draw out some cerebrospinal fluid. The fluid is examined for leukemia cells.

Lab tests: Doctors use a number of very precise lab tests to diagnose and classify leukemia.

Blood cell counts and other blood tests: Changes in the numbers of different blood cell types and how the cells look under a microscope can suggest leukemia. Most people with acute lymphocytic leukemia (ALL) have too many white blood cells, not enough red cells, and not enough platelets. Also, many of the white cells will be blasts, a type of immature cell not normally found in circulating blood. These cells don't work the way they should.

People already known to have leukemia will have tests done to measure the amount of certain chemicals in the blood. These tests do not tell if they have leukemia but can help tell how well their kidneys and liver are working.

A doctor with special training in blood diseases looks at all of the biopsy samples (bone marrow, lymph node tissue, blood, and cerebrospinal fluid) under a microscope. The doctor looks at the size and shape of the cells as well as other features to classify the cells into specific types. An important goal of this process is to see whether the cells appear mature or not. The most immature cells are called blasts. The number of blasts in the bone marrow is important in telling if a person has leukemia.

Having at least 20% to 30% of blasts in the marrow is generally the benchmark for a diagnosis of ALL.

Other special tests which look at blood, marrow, and even DNA to help tell which type of leukemia a person has. These are complex medical and chemical tests. Your doctor can tell you which of these you might need.

Imaging studies: Imaging studies are ways of producing pictures of the inside of the body.

Because leukemia does not usually form tumors, imaging tests are not always helpful. Imaging studies might be done in people with ALL, but they are done more often to look for infections or other problems rather than for the leukemia itself.

• X-rays may be done to see if there is a lung infection. The x-ray can also show enlarged lymph nodes in the chest.
• CT (computed tomography) scans are special kinds of x-rays in which a beam moves around the body, taking pictures from different angles. The pictures are combined by a computer into an image of a slice of the body. CT scans are helpful in looking at internal organs. They can show pockets of infection, enlarged organs, and any large collection of leukemia cells.
• MRI (magnetic resonance imaging) is a method that uses powerful magnets and radio waves to produce detailed, computer-generated pictures of the body. MRI scans are helpful in looking at the brain and spinal cord. MRI scans take longer than CT scans. Also, you may be placed inside a tube, which can feel confining.
• Gallium scans and bone scans involve injecting a radioactive chemical into the blood. The chemical collects in areas of cancer or infection, where it can be seen by a special camera. These tests are useful when a person has bone pain that might be caused by either infection or cancer involving bones.
• Ultrasound uses sound waves to produce images of internal organs. This test can tell solid from fluid-filled masses. It can help to show whether the kidneys, liver, or spleen are enlarged. This is an easy test to have done. You simply lie on a table and a kind of wand is moved over the part of your body being examined.

How is Acute Lymphocytic Leukemia Classified?
 
Most types of cancer are assigned a numbered stage based on the size of the tumor and how far it has spread. But there is no need to stage leukemia in this way because it already involves all the bone marrow and, in many cases, it has also spread to other organs.

Several years ago, an international conference of doctors who specialize in leukemia was held to decide on the best system for classifying acute leukemia. They decided upon 3 subtypes for ALL.

For leukemia, lab tests focus on finding out the exact type and subtype of leukemia, which in turn helps the doctor predict which treatments will work best.

Certain features of the disease separate patients who are likely to have a good response to treatment from those likely to have a poor response. These are called prognostic factors. These features include the patient's age, white blood cell count, certain test results, and initial response to chemotherapy.

How Is Acute Lymphocytic Leukemia Treated?
 
As noted before, acute lymphocytic leukemia is not a single disease. It is really a group of diseases and people with different subtypes vary in how they respond to treatment. Treatment options are based on the subtype as well as on the prognostic features.

• Chemotherapy is the major treatment for ALL.
• Surgery and radiation may be used in some cases.

Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they spread throughout the body. Chemotherapy for ALL involves the use of several drugs given over a long period of time.

Side Effects of Chemotherapy

While chemotherapy drugs kill cancer cells, they can also damage normal cells. This happens because they target rapidly growing cells such as cancer cells but in the process they also damage other fast growing cells.

The side effects of chemotherapy depend on the type and dose of drugs given and the length of time they are taken. These side effects might include:

• hair loss
• mouth sores
• higher risk of infection (due to low white blood cells)
• easy bruising or bleeding (due to low blood platelets)
• tiredness (due to low red blood cells)
• loss of appetite
• nausea
• vomiting

The side effects usually go away after treatment ends. Be sure to talk to your doctor if you are having trouble with side effects because there are often ways to manage them during treatment. For example, there are drugs than can be taken along with the chemotherapy to prevent or reduce nausea and vomiting. Drugs known as growth factors are sometimes given to keep blood counts higher and reduce the chance of infection.

Other ways you can reduce the risk of infection are by avoiding exposure to germs as much as possible and by carefully washing your hands and not eating uncooked fruits and vegetables. While in treatment you should also avoid large crowds and people who are sick.

During and after treatment, you might also get antibiotics as added protection. If your platelet counts are low, you might get platelet transfusions to protect against bleeding. Low red blood cell counts, causing shortness of breath and tiredness, can be treated with drugs or with transfusions.

Tumor lysis syndrome is a side effect caused by the rapid breakdown of leukemia cells due to treatment. When these cells die, they release substances into the bloodstream that can affect the kidneys, heart, and nervous system. Extra fluids or certain drugs that help rid the body of these toxins can help prevent this problem.

Finally, some people treated for ALL could later develop AML (acute myeloid leukemia). Less often, people cured of leukemia might later develop non-Hodgkin lymphomas or other cancers.

Targeted Therapy

A newer drug called imatinib (Gleevec) has been used to successfully treat chronic myeloid leukemia (CML). Clinical trials are going on now to see if this drug will be helpful in treating some people with ALL as well. Early reports have shown a better outcome when imatinib was used. This drug also seems to cause fewer side effects than other chemotherapy drugs. Possible side effects include diarrhea, nausea, muscle pain, and fatigue, but these are often mild.

Monoclonal antibodies are large proteins made in the lab. They attach to certain molecules on the surface of leukemia cells. These antibodies have been used to treat lymphomas. Researchers are now looking at whether they might be useful in treating some patients with ALL. Early results have been favorable, but it’s still too early to know for sure.

Surgery

Surgery is not generally used to treat leukemia because this is a disease of blood and bone marrow and it is not possible cure it with surgery. But surgery may be used to help deliver treatment. A plastic tube can be placed into a large vein. The tube, called a venous access device, allows chemotherapy drugs or other medicines to be given and blood samples removed without the need for many needle sticks. The patient will need to learn how to take care of the device to prevent it from getting infected.

Radiation Therapy

Radiation therapy is the use of high energy x-rays to kill cancer cells. It is sometimes used to treat leukemia that has spread to the brain and spinal cord or to the testicles.

Radiation to several parts of the body is often done before a bone marrow or blood stem cell transplant (see below). It is also used, though rarely, in an emergency to shrink a mass if it is pressing on the windpipe. But more often chemotherapy is used instead.

Bone Marrow or Peripheral Blood Stem Cell Transplantation

As noted earlier, chemotherapy can harm normal cells as well as cancer cells.

Stem cell transplantation (SCT) offers a way for doctors to use high doses of chemotherapy. Although the drugs destroy the patient's bone marrow, transplanted stem cells can restore the bone marrow’s ability to make blood. Transplants using the patient’s own stem cells are sometimes used for people with acute leukemia that is in remission.

The treatment works like this:

• stem cells for are collected from the bone marrow, or from the bloodstream in a process called apheresis.
• These stem cells can come from either the patient or from a suitable donor.
• The stems cells are frozen and stored.
• Patients are then given very high doses of chemotherapy to kill the cancer cells.
• They also receive total body radiation to kill any remaining cancer cells.
• After treatment, the stored stem cells are given to the patient as a blood transfusion.
• Then the waiting period begins as the stem cells settle in the patient's bone marrow and start to grow and produce blood cells.

People who receive a donor's stem cells are given drugs to prevent rejection as well as other medicines as needed to prevent infections. Usually around 10 to 21 days after the stem cells are given, they start making new white blood cells. Then they begin making platelets, and finally, red blood cells.

Patients having SCT have to be kept away from germs as much as possible until their white blood cell count is at a safe level. They are kept in the hospital until the white cell count reaches a certain number, usually around 1,000. After they go home, they will be seen in the outpatient clinic almost every day for several weeks.

Stem cell transplantation is still a fairly new and complex treatment. If the doctors think that a person with leukemia might be helped by this treatment, it is important that it be done at a hospital where the staff has experience with the procedure. Some transplant programs may not have experience in certain transplants, especially those from unrelated donors.

Stem cell transplantation is very expensive and requires a long hospital stay. Because some insurance companies see it as an experimental treatment, they might not pay for it.

Side Effects of Stem Cell Transplantation

Side effects from stem cell transplantation can be divided into early and long-term effects.

The early side effects are basically the same as those caused by any other type of high-dose chemotherapy. But other side effects can last for a long time, or they may not occur until years after the transplant.

These long-term side effects can include the following:

• radiation damage to the lungs, causing shortness of breath
• graft-versus-host disease (GVHD), which occurs only in a donor transplant
• damage to the ovaries causing infertility and the loss of menstrual periods
• damage to the thyroid gland that causes problems with changing food into energy
• cataracts (damage to the eye that can affect vision)
• bone damage (if damage is severe, the patient will need to have part of the bone and joint replaced.)
• Graft-versus-host disease is the main problem of a donor stem cell transplant. It happens when the immune system of the patient is taken over by that of the donor. The donor immune system then starts to attack the patient’s other tissues and organs.

Symptoms can include

• severe skin rashes with itching 
• severe diarrhea.
• The liver and lungs may also be damaged.
•  The patient may also become tired and have aching muscles.
• If severe enough, the disease can be fatal.

Drugs that affect the immune system may be given to try to control it. On the plus side, this disease also causes any remaining leukemia cells to be killed by the donor immune system.

For ALL, chemotherapy treatments are given in the following phases:

Remission induction: The purpose of the first phase is to bring about a remission--the disappearance of the signs and symptoms of the cancer. A remission may not be a cure.

Consolidation: The goal of this phase is to get rid of leukemia cells from places where they can "hide." This lasts from one to a few months.

Maintenance: Once the number of leukemia cells has been reduced by the first 2 phases of treatment, this last phase can begin. Maintenance, which usually consists of lower doses of chemotherapy drugs, lasts about 2 years.

In general, about 8 out of 10 patients will have a complete response to these treatments. That means that leukemia can no longer be seen in their bone marrow. But about half of these patients will have a relapse, so the overall cure rate is around 30%.

Central nervous system treatment: Because ALL often spreads to the coverings of the brain and spinal cord, patients often receive chemotherapy in the spinal fluid or radiation therapy of the head as a method of prevention.

Finally, some patients who are at high risk for relapse (because of poor prognostic factors) may have a stem cell transplant.

What if the Leukemia Doesn’t Respond or Comes Back After Treatment?

If the leukemia comes back after treatment, it will most often do so in the bone marrow and blood. Once in a while, the brain or spinal fluid will be the first place it returns.

If the leukemia had gone away and has now come back, it may be possible to bring about another remission, although most doctors think this remission will be only temporary. In these cases most doctors will consider a stem cell transplant.

If the leukemia keeps coming back or doesn’t go away, chemotherapy will finally not be very helpful. If a stem cell transplant is not an option, entering a clinical trial (see section on Clinical Trials) might be a good idea.

If neither of these options is the right one, then it may be time to focus on relieving symptoms. The doctor may suggest more mild chemotherapy to slow the growth of the leukemia. If there is pain, then it’s important to treat it with pain killing medicines. Sometimes medicines or blood transfusions are needed to correct low blood counts and tiredness. If depression is a problem, that can be treated as well. Nausea and loss of appetite can be helped by high-calorie food supplements and medicines. Antibiotics may be needed to treat infection.

What Is Acute Myeloid Leukemia (AML)
 
Leukemia is a type of cancer that starts in the soft, inner part of the bones (bone marrow) but often moves quickly into the blood. It can then spread to other parts of the body, such as the lymph nodes, the spleen, liver, central nervous system and other organs. Both children and adults can get leukemia.

"Acute" means that the cancer develops quickly, and if not treated, could be fatal in a few months. Acute myeloid leukemia (AML) is a type of cancer that starts from cells that normally develop into blood cells.

Leukemia is a complex disease with many different types and sub-types. The kind of treatment given and the outlook for the person with leukemia vary greatly according to the exact type and other factors.

How Many People Get Acute Myeloid Leukemia?
 
In 2006 there will be about 35,070 new cases of all types of leukemia in the United States. About half of these will be acute leukemias. There will be about 11,930 new cases of acute myelogenous leukemia (AML). Nine out of 10 cases of AML are found in adults. There will be about 9,040 deaths from AML in the United States this year. The average age of a patient with AML is 65. AML is slightly more common among men than among women.

AML can be kept in remission for a long time or cured in some adults. Depending on certain features of the leukemia cells, some patients have a better and some a worse outlook (prognosis). Overall, the 5-year survival rate in adults under 65 is about 33%. In people over 65 it is 4%. The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is found. Of course, many people live much longer than 5 years.

What Causes Acute Myeloid Leukemia?
 
A risk factor is something that increases a person's chance of getting a disease. Some risk factors, like smoking, can be controlled. Others, such as a person's age, can't be changed.

• Smoking is a proven risk factor for AML. Although many people know that smoking causes lung cancer, few realize that it can affect cells that do not come into direct contact with smoke. Cancer-causing substances in tobacco smoke get into the bloodstream and spread to many parts of the body. Smoking causes about 1 in 5 cases of AML.
• long-term exposure to high levels of benzene
• high-dose radiation exposure (such as from an atomic blast or nuclear reactor accident)
• People who have had other cancers and were treated with certain chemotherapy drugs are more likely to develop AML. Most of these cases of AML happen within 9 years after treatment.
• There is some concern about very high-voltage power lines as a risk factor for leukemia. The National Cancer Institute has several large studies going on now to look into this question. So far, the studies show either no increased risk or a very slightly increased risk. Clearly, most cases of leukemia are not related to power lines.

Can Acute Myeloid Leukemia Be Prevented?
 
Most people who develop leukemia do not have any of the above risk factors. The cause of their leukemia remains unknown at this time. Because the cause is not known, there is no way to prevent most cases of leukemia. There is one important exception: smoking. About 20% of adult acute myeloid leukemia cases are linked to smoking.
 
How Is Acute Myeloid Leukemia Found?
 
At this time, there are no special tests that can find acute myeloid leukemia early. The best course of action is to report any symptoms to the doctor right away.

Leukemia can cause many signs and symptoms, some of them very general in nature. Keep in mind that the symptoms mentioned below are most often caused by something other than cancer.

General symptoms of AML can include:

• weight loss
• fever
• loss of appetite
• anemia 
• increased risk of infection
• bruising
• bleeding
• frequent or severe nosebleeds
• bleeding from the gums
• bone pain or joint pain

Most symptoms of acute leukemia are caused by a shortage of normal blood cells, a result of the crowding out of normal blood cell-producing bone marrow by leukemia cells. As a result, the person doesn't have enough properly working red blood cells, white blood cells, and platelets. Anemia is a result of a shortage of red blood cells. Anemia causes shortness of breath, fatigue, and a pale skin color.

Not having enough normal white blood cells can increase the risk of infection. Although people with leukemia may have very high white blood cell counts, the cells are not normal and do not protect against infection very well.

Not having enough blood platelets can lead to bruising, bleeding, frequent or severe nosebleeds, and bleeding from the gums.

Some people have bone pain or joint pain caused by the spread of cancer cells to the surface of the bone or into the joint. Leukemia can also cause swelling of the liver and spleen. If the disease has spread to the lymph nodes, these nodes may be swollen. One type of AML is prone to spread to the gums, causing them to swell, become painful, and bleed. Spread to the skin can cause small colored spots that look like common rashes.

AML can spread outside of the bone marrow to other organs. If it spreads to the brain or spinal cord (central nervous system), it can cause a variety of symptoms such as headaches, weakness, seizures, vomiting, trouble keeping one's balance, and blurred vision. This is not common in people with AML.

What Tests will be Done If Acute Myeloid Leukemia Is Suspected ?
 
If there is reason to think that you might have leukemia, the doctor will need to take samples of cells from your blood and bone marrow to find out if the disease is really present.

Blood cell counts and other blood tests: Changes in the numbers of different blood cell types and how the cells look under a microscope can suggest leukemia. Most people with AML will have too many white blood cells, not enough red cells, and not enough platelets. Also, many of the white cells will be blasts, a type of immature cell not normally found in circulating blood. These cells don't work the way they should.

People already known to have leukemia will have tests done to measure the amount of certain chemicals in the blood. These tests do not tell if they have leukemia, but can help tell how well their kidneys and liver are working.

Bone marrow tests: In bone marrow aspiration, a thin needle is used to draw up a small amount of liquid bone marrow. During a bone marrow biopsy, a small cylinder of bone and marrow (about ½ inch long) is removed with a slightly larger needle.

Both samples are usually taken at the same time from the back of the hipbone.

• The patient lies on his or her side and the area is cleaned with a special soap.
• Before the sample is taken, the doctor injects an anesthetic into the area near the back of the hipbone to numb it.
• Then the doctor makes a small cut in order to insert a needle.
• The needle is moved through the bone with a twisting motion.
• Sometimes the needle going into the bone is painful, but it only lasts a short time.
• During aspiration, the sucking out is often painful for a brief moment.

These tests are used to tell whether leukemia is present and also, if you are having treatment, how well the disease is responding.

Lumbar puncture: In this procedure, a small needle is placed into the spinal cavity in the lower back to draw out some cerebrospinal fluid. The fluid is examined for leukemia cells. This test is not usually done for people with AML.

Lab tests: Doctors use a number of very precise lab tests to diagnose and classify leukemia.

A doctor with special training in blood diseases examines all of the biopsy samples (bone marrow, blood, and cerebrospinal fluid) under a microscope. The doctor looks at the size and shape of the cells as well as other features to classify the cells into specific types. An important goal of this process is to see whether the cells appear mature or not. The most immature cells are called blasts. The number of blasts in the bone marrow is important in telling if a person has leukemia.

Other special tests, such as flow cytometry, may also help tell which type of leukemia a person has.

Imaging studies: Imaging studies are ways of producing pictures of the inside of the body. There are several kinds of these studies that might be done in people with leukemia. They are done most often to look for infections or other problems rather than for the leukemia itself.

• X-rays may be done if the doctor thinks there might be a lung infection.
• CT (computed tomography) scans are special kinds of x-rays in which a beam moves around the body, taking pictures from different angles. The pictures are combined by a computer into an image of a slice of the body. CT scans are helpful in looking at internal organs. They can show pockets of infection, enlarged organs, and any large collection of leukemia cells.
• MRI (magnetic resonance imaging) is a method that uses powerful magnets and radio waves to produce detailed, computer-generated pictures of the body. MRI scans are helpful in looking at the brain and spinal cord. MRI scans take longer than CT scans. Also, you may be placed inside a tube, which can feel confining.
• Gallium scans and bone scans involve injecting a radioactive chemical into the blood. The chemical collects in areas of cancer or infection, where it can be seen by a special camera. These tests are useful when a person has bone pain that might be caused by either infection or cancer involving bones.
• Ultrasound uses sound waves to produce images of internal organs. This test can tell solid from fluid-filled masses. Ultrasound is also used to show whether the kidneys, liver, or spleen have been affected by leukemia. This is an easy test to have done. You simply lie on a table and a kind of wand is moved over the part of your body being examined.

How Is Acute Myeloid Leukemia Classified?
 
Most types of cancer are assigned a numbered stage based on the size of the tumor and how far it has spread. But there is no need to stage leukemia in this way because it already involves all the bone marrow and, in many cases, it has also spread to other organs. Lab tests focus on finding out the exact type (and subtype) of leukemia. This in turn helps predict which treatments will work best and the outlook (prognosis) for the patient.

Several years ago, an international conference of doctors who specialize in leukemia was held to decide on the best system for classifying acute leukemia. They decided upon 8 subtypes for AML, labeled M0 through M7, based on the type of cell from which the leukemia started. This system (the French-American-British or FAB system) was based at first on how the cells looked under the microscope. But now doctors use many advanced tests to classify AML.

Some subtypes of AML produce certain symptoms. For example, bleeding or blood clotting problems often happen to people with the M3 subtype of AML. Finding out if a person has M3 leukemia is very important for 2 reasons. First, serious problems can often be prevented by the right treatment. Second, M3 leukemias usually respond to certain drugs related to vitamin A. Using these drugs allows doctors to use lower doses of chemotherapy drugs and thus reduce side effects.

Certain features of the disease separate patients who are likely to have a good response to treatment from those likely to have a poor response. These are called prognostic factors. These factors include the patient's age, white blood cell count, certain test results, response to chemotherapy, and whether or not the person had been treated earlier for another cancer.

While some patients might find detailed information about subtypes and prognostic factors helpful, others may find it a bit overwhelming. The American Cancer Society has more information about these subtypes and prognostic factors in a separate document, available through our toll-free number or on our Web site.

How Is Acute Myeloid Leukemia Treated? 
 
As noted before, acute myeloid leukemia is not a single disease. It is really a group of diseases, and people with different subtypes vary in how they respond to treatment. Treatment options are based on the subtype as well as on the prognostic features. Several different types of treatment may be used in people with AML.

Treatment of AML is divided into 2 phases:

• remission induction
• post-remission therapy

Remission induction: This first phase, which is meant to destroy most of the normal and leukemic bone marrow cells, usually lasts one week. During this time and in the weeks right after, the patient's blood cell counts will be very low. The doctor will take measures to protect against complications. If 1 week of treatment fails to bring about a remission, the process is repeated 1 or 2 more times. Induction works for about 6 or 7 out of 10 patients.

Consolidation (post-remission) therapy: While the first phase of treatment usually destroys nearly all of the cancer cells, there may still be some "hidden" leukemic cells. The purpose of the second phase is to destroy these cells and prevent a relapse. The options for post-remission treatment are either further chemotherapy or stem cell transplantation, described later in this article. Four years after this treatment, 40% of young patients (younger than 60 years) will not show any signs of leukemia. In older adults, this number is around 15%.

Doctors look at several factors when recommending post-remission treatment for a patient. These factors include:

• how much chemotherapy it took to bring about a remission
• whether there is a stem cell donor (brother or sister or unrelated donor) who matches the patient’s tissue type
• whether it looks as if leukemia-free bone marrow stem cells can be collected from the patient
• whether there are good or poor prognostic factors
• the patient’s age
• what the patient prefers

Maintenance therapy: In the past it was thought that a short burst of intense therapy as described above was the best way to treat AML. But a recent study from Germany of over 800 patients found that patients had better outcomes if they also received low doses of chemotherapy for 3 years after their induction and consolidation courses.

Surgery

Surgery is not generally used to treat leukemia because this is a disease of blood and bone marrow and it is not possible to bring about a cure with surgery. But surgery may be used to help deliver treatment. A plastic tube can be placed into a large vein. The tube, called a venous access device, allows chemotherapy drugs or other medicines to be given and blood samples removed without the need for many needle sticks. The patient will need to learn how to take care of the device to prevent it from getting infected.

Radiation Therapy

Radiation therapy, the use of high energy x-rays to kill cancer cells, has a limited role to play in the treatment of people with leukemia.

For adults with acute leukemia, radiation might be used to treat cancer that has spread beyond the bone marrow. It might also be used, though rarely, in an emergency to treat pressure on the windpipe. But more often chemotherapy is used instead.

Chemotherapy

Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they spread throughout the body. The first chemotherapy for AML is a combination of 2 or 3 drugs given over about a week at the hospital.

Treatment of M3 Leukemia

Patients with M3 leukemia can develop serious blood clotting or bleeding problems. This can usually be prevented or treated by giving them a blood thinner. Other treatments might include transfusions of platelets or other blood products.

The treatment of M3 leukemia differs from usual AML treatment because a non-chemotherapy drug known as ATRA (related to vitamin A) is also used. For best results, ATRA is combined with other chemotherapy drugs. Another drug, arsenic trioxide, has been effective in treating patients with APL whose leukemia comes back after the first treatment

Side effects from ATRA can include retinoic acid syndrome. This very serious side effect can cause breathing problems from fluid buildup, low blood pressure, and kidney damage.

After remission, further treatment consists of 2 or more courses of chemotherapy followed by maintenance with ATRA for a least one year. About 70% to 90% of patients are cured with this approach.

Side Effects of Chemotherapy

While chemotherapy drugs kill cancer cells, they can also damage normal cells. This happens because they target rapidly growing cells such as cancer cells, but in the process they also damage other fast growing cells.

Bone marrow, the lining of the mouth and intestines, and hair follicles all grow rapidly and are likely to be affected by chemotherapy. As a result, people being treated with chemotherapy may have some side effects:

• higher risk of infection (from low white blood cell counts)
• bruising or bleeding easily (from low blood platelets)
• tiring easily (from low red blood cell counts)
• hair loss
• nausea
• vomiting
• loss of appetite

These side effects usually go away shortly after treatment ends. There are often ways to manage these side effects during treatment. For example, there are drugs than can be taken along with the chemotherapy to prevent or reduce nausea and vomiting. Drugs known as growth factors are sometimes given to keep blood counts higher and reduce the chance of infection.

Other ways you can reduce the risk of infection are by avoiding exposure to germs as much as possible and by carefully washing your hands and not eating uncooked fruits and vegetables. While in treatment you should also avoid large crowds and people who are sick. It’s best to also avoid fresh flowers and plants because they carry mold.

During and after treatment, you might also get antibiotics as added protection. If your platelet counts are low, you might get platelet transfusions to protect against bleeding. Low red blood cell counts, causing shortness of breath and tiredness, can be treated with drugs or with transfusions.

Tumor lysis syndrome is a side effect caused by the rapid breakdown of leukemia cells due to treatment. When these cells die, they release substances into the bloodstream that can affect the kidneys, heart, and nervous system. Extra fluids or certain drugs that help rid the body of these toxins can help prevent this problem.

Organs that could be damaged by chemotherapy include the kidneys, liver, testes, ovaries, brain, heart, and lungs. By watching the patient carefully, the doctor may be able to prevent many of these side effects. If serious side effects happen, though, the drugs may have to be reduced or stopped—at least for a while.

Monoclonal Antibodies

These are proteins made in the lab and designed to attach to certain molecules on the surface of AML cells. Some of these antibodies have radioactive chemicals or cell poisons attached to them so that when they are injected into the patient, they attach to the cancer cells and kill them. One such antibody (Mylotarg) has recently been approved for use in older adults with AML who have relapsed after treatment or who might not be able to take the side effects of further chemotherapy. Studies are still going on to look at the best use of this approach.

Bone Marrow or Peripheral Blood Stem Cell Transplantation

As noted earlier, chemotherapy can harm normal cells as well as cancer cells. Stem cell transplantation (SCT) offers a way for doctors to use high doses of chemotherapy. Although the drugs destroy the patient's bone marrow, transplanted stem cells can restore the bone marrow’s ability to make blood. Transplants using the patient’s own stem cells are sometimes used for people with acute leukemia that is in remission.

The treatment works like this:

• stem cells for are collected from the bone marrow or from the bloodstream in a process called apheresis.
• These stem cells can come from either the patient or from a suitable donor.
• The stem cells are frozen and stored.
• The patient is then given very high doses of chemotherapy to kill the cancer cells.
• They also receive total body radiation to kill any "hidden" cancer cells.
• After treatment, the stored stem cells are given to the patient as a blood transfusion.
• Then the waiting period begins as the stem cells settle in the patient's bone marrow and start to grow and produce blood cells.

People who receive a donor's stem cells are given drugs to prevent rejection as well as other medicines as needed to prevent infections. Usually around 10 to 21 days after the stem cells are given they start making new white blood cells. Then they begin making platelets, and finally, red blood cells.

Patients having SCT have to be kept away from germs as much as possible until their white blood cell count is at a safe level. They are kept in the hospital until the white cell count reaches a certain number, usually around 1,000. After they go home, they will be seen in the outpatient clinic almost every day for several weeks.

Stem cell transplantation is still a fairly new and complex treatment. If the doctors think that a person with leukemia might be helped by this treatment, it is important that it be done at a hospital where the staff has experience with the procedure. Some transplant programs may not have experience in certain transplants, especially those from unrelated donors.

Stem cell transplantation is very expensive and requires a long hospital stay. Because some insurance companies see it as an experimental treatment, they might not pay for it.

Side Effects of Stem Cell Transplantation

Side effects from SCT can be divided into early and long-term effects. Early side effects are basically the same as those from any other type of high-dose chemotherapy. They are caused by damage to the bone marrow and other rapidly growing tissues of the body.

But other side effects can go on for a long time. Sometimes they don't show up until years after the transplant. The long-term side effects could include the following:

• radiation damage to the lungs, causing shortness of breath
• graft-versus-host disease, which happens only in donor transplants. This serious side effect can occur when the donor's immune system cells attack tissues of the patient's skin, liver, mouth, or other organs. Symptoms include weakness, fatigue, dry mouth, rashes, infection, and muscle aches.
• damage to the ovaries causing infertility and loss of menstrual periods
• damage to the thyroid gland that causes problems with metabolism
• cataracts (damage to the lens of the eye)
• bone damage; if damage is severe, the patient will need to have part of the bone and joint replaced.
• Graft-versus-host disease is the main problem of a donor stem cell transplant. It happens when the immune system of the patient is taken over by that of the donor. The donor immune system then starts to attack the patient’s other tissues and organs.

Symptoms can include severe skin rashes with itching and severe diarrhea. The liver and lungs may also be damaged. The patient may also become tired and have aching muscles. If severe enough, the disease can be fatal. Drugs that affect the immune system may be given to try to control it. On the plus side, this disease also causes any remaining leukemia cells to be killed by the donor immune system. So, sometimes the doctors want to see some graft-versus-host disease occur.

What if the Leukemia Doesn’t Respond or Comes Back After Treatment?

If the disease comes back it will most often be in the bone marrow and blood. Sometimes the brain or spinal fluid will be the first place it is seen. This will be treated with chemotherapy given directly into the spinal fluid. If the leukemia never went away with the first treatment, then more treatment is not likely to work, even with new drugs. If the leukemia did go away and has now come back, another remission might be possible, although most doctors think it would be only temporary. They might suggest a stem cell transplant in this case.

If the leukemia keeps coming back or doesn’t go away, chemotherapy will finally not be very helpful. If a stem cell transplant is not an option, entering a clinical trial (see section on Clinical Trials) might be a good idea.

If neither of these options is the right one, then it may be time to focus on relieving symptoms. The doctor may suggest more mild chemotherapy to slow the growth of the cancer. If there is pain, then it’s important to treat it with pain killing medicines. Sometimes medicines or blood transfusions are needed to correct low blood counts and tiredness. If depression is a problem, that can be treated as well. Nausea and loss of appetite can be helped by high-calorie food supplements and medicines. Antibiotics may be needed to treat infection.

What is Chronic Lymphocytic Leukemia?
 
Chronic lymphocytic leukemia (CLL) is a type of cancer that starts in lymphocytes (white blood cells) of the bone marrow. It then invades the blood. It can spread to the lymph nodes, the spleen, liver, and other parts of the body.

Doctors have learned that there are probably 2 different kinds of CLL.

• One kind is very slow growing and rarely needs to be treated. The average survival for people with this kind of CLL is around 13 to 15 years.
• Another kind is a faster growing and more serious disease. People with this form of CLL have an average survival of only about 6-8 years. The leukemia cells from these 2 types look alike. But certain new lab tests can tell them apart

Hairy cell leukemia (HCL), like CLL, is a cancer of the lymphocytes that grows slowly. But HCL is often thought of differently from CLL because of differences in symptoms and treatment.

How Many People Get Chronic Lymphocytic Leukemia?
 
The American Cancer Society estimates that, in 2006, there will be about 10,020 new cases of chronic lymphocytic leukemia (CLL) in the US. About 4,660 people in the US will die of CLL during 2006.

Chronic lymphocytic leukemia affects only adults. The average age of patients is about 70. It is rarely seen in people under the age of 40.
 
What Causes Chronic Lymphocytic Leukemia? 

There are no known risk factors for CLL that are linked to lifestyle, but there are a very few factors in the environment that are linked to chronic leukemia.

• chemicals used in farming, such as herbicides and insecticides, may increase the risk of CLL.
• Exposure to Agent Orange, an herbicide used during the Vietnam War, has been linked to an increased risk of CLL.
• The only known inherited risk factor for chronic leukemia is having first-degree relatives (parents, siblings, or children) who have had CLL.

Can Chronic Lymphocytic Leukemia Be Prevented?

Although many types of cancer can be prevented by lifestyle changes to avoid certain risk factors, there are no known risk factors for CLL that a person can change. So right now there is no way to prevent CLL from developing.

How Is Chronic Lymphocytic Leukemia Found?
 
At this time, there are no special tests that can find chronic leukemia early. The best course of action is to report any symptoms to the doctor right away. These symptoms are discussed in the section that follows.

Symptoms of CLL

Many people with CLL have no symptoms at the time their cancer is found. In these cases, the cancer is found by blood tests done for some other reason. Even when there are symptoms, they may be very general. Many of the symptoms of advanced CLL happen because the leukemia cells replace the bone marrow’s blood-producing cells. As a result, people do not have enough blood cells and platelets that are working as they should.

• fatigue
• weakness
• weight loss
• fever
• bone pain
• anemia
• increased risk of infection. Although people with leukemia may have very high white blood cell counts, the cells are not normal and do not protect against infection very well.
• Not having enough blood platelets can lead to bruising, bleeding, frequent or severe nosebleeds, and bleeding from the gums.
• Some people have bone pain or joint pain caused by the spread of cancer cells to the surface of the bone or into the joint.
• Leukemia can also cause swelling of the liver and spleen. If the disease has spread to the lymph nodes, these nodes may be swollen.

What Tests will I have to Look for Leukemia?

Bone marrow tests: In bone marrow aspiration, a thin needle is used to draw up a small amount of liquid bone marrow. The skin and the surface of the bone are first numbed, but the test can still be uncomfortable. During a bone marrow biopsy, a small cylinder of bone and marrow (about ½" long) is removed with a slightly larger needle.

Both samples are usually taken at the same time from the back of the hipbone. These tests are used to tell whether leukemia is present and also, if the person is having treatment, how well the disease is responding.

Excisional lymph node biopsy: For this test, an entire lymph node is removed. If the node is near the skin's surface, a simple operation can be done by numbing just the area around the node. But if the node is inside the chest or abdomen, the patient will need general anesthesia. This procedure is only rarely needed for people with CLL unless a lymph node has grown very large.

Lumbar puncture (spinal tap): During this test, a small needle is placed into the spinal cavity in the lower back to draw out some cerebrospinal fluid. The fluid is looked at for leukemia cells. This test is only done for patients with CLL if it appears that there may be leukemia cells in their brain or spinal cord or an infection in those areas.

Blood cell counts and blood cell examination: Changes in the numbers of different blood cell types and how the cells look under a microscope can suggest leukemia. Most people with CLL have too many of the white blood cells called lymphocytes. Anything more than 10,000 lymphocytes in a cubic millimeter of blood makes CLL almost certain. Normal is less than 5,000. Often there are too few red blood cells and blood platelets as well.

People with leukemia will have tests done to measure the amount of certain chemicals in the blood. These tests do not tell if they have leukemia but can help tell how well their kidneys and liver are working. The test results also help the doctor decide whether treatment is needed to correct low or high blood levels of certain minerals.

Lab Tests for CLL

Any samples taken (blood, bone marrow, lymph nodes, etc.) are looked at under a microscope by a doctor with special training (pathologist). The doctor looks at the size and shape of the cells as well as other features to classify the cells into specific types. An important goal of this process is to see whether the cells appear mature or not. The most immature cells are called blasts. These blasts are not effective in fighting infections, but they can reproduce, crowding out normal, mature cells. Doctors also look at whether there are the normal numbers of blood forming cells or whether leukemia cells have replaced these.

In addition, doctors use a number of very precise lab tests to diagnose and classify leukemia. You might hear some of the following terms used: cytochemistry, flow cytometry, immunocytochemistry, cytogenetics, and molecular genetic studies.

Imaging Studies

Imaging studies are ways of taking pictures of the inside of the body. There are several methods that might be used for people with leukemia.

• Chest x-rays: These can be used to find large lymph nodes in the chest.
• CT (computed tomography) scans: These are special kinds of x-rays in which a beam moves around the body, taking pictures from different angles. CT scans can show better than an x-ray whether lymph nodes in the chest or in other parts of the body are enlarged. CT scans take longer than regular x-rays. You will need to lie still on a table. The part of your body being scanned is placed within the scanner. The test is painless, but you will need to lie still for minutes at a time.
• MRI (magnetic resonance imaging): This test uses powerful magnets and radio waves to produce detailed, computer-generated pictures of the body. MRI scans are very helpful in looking at the brain and spinal cord. They take longer than CT scans, often up to an hour. You may need to lie inside a tube for the test.
• Ultrasound: This test uses sound waves to produce images of internal organs. Ultrasound can look for lymph nodes inside the abdomen. This is a very easy test to take. You simply lie on a table and a small wand (transducer) is moved over the part of the body being looked at.

How Is Chronic Lymphocytic Leukemia Staged?
 
Most types of cancer are assigned a stage based on the size of the tumor and how far it has spread. This system generally does not apply to leukemia because leukemia does not usually form a solid mass or tumor.

Also, leukemia involves the bone marrow and, in many cases, has already spread to other organs in the body when it is found. For CLL, lab tests focus on giving a clear description of different features of the disease. These features, in turn, help predict the likely outcome for the patient and help to guide treatment decisions.

There are 2 different systems for staging CLL.

• The Rai system is the one used more often in this country. In the Rai system there are 5 stages from 0 to IV (0 to 4). These stages can also be used to divide patients into low-, intermediate-, or high-risk groups.
• The Binet system uses the letters A, B, and C to define the stages.

Other factors besides the stage can help predict a patient’s outlook for survival. These factors are called prognostic factors . Factors that are linked to shorter survival time are called adverse prognostic factors. Those linked to longer survival are favorable prognostic factors. Your doctor can tell you if any of these factors apply in your case and what they mean.

Recently doctors have come to think that there are probably 2 kinds of CLL. One kind has a very good outlook and the other a less favorable one. New tests help the doctor tell these two types apart. People with the poorer outlook have higher amounts of certain substances in their cells. Most experts feel that these new tests will be more important than the staging systems mentioned above because they are more useful for the individual patient.

How Is Chronic Lymphocytic Leukemia Treated? 
 
Chemotherapy

Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or the cerebrospinal fluid or taken by mouth. Once the drugs enter the bloodstream, they reach all parts of the body. Chemotherapy is useful for cancers like leukemia that spread throughout the body. Treatment for leukemia often involves a combination of several drugs given over a period of time.

While chemotherapy drugs kill cancer cells, they can also damage normal cells. This happens because they target rapidly growing cells such as cancer cells but in the process they also damage other fast growing cells. This can lead to side effects.

Cells in the bone marrow, the lining of the mouth and intestines, and hair follicles all grow rapidly and are likely to be damaged by chemotherapy. As a result, side effects of chemotherapy can include short-term hair loss, nausea, vomiting, and loss of appetite. People being treated with chemotherapy may also have a higher risk of infection (due to low white blood cell counts), may bruise or bleed easily (from low blood platelets), and tire easily (from low red blood cell counts).

These side effects usually go away shortly after treatment ends. There are often ways to manage these side effects during treatment. For example, there are drugs than can be taken along with the chemotherapy to prevent or reduce nausea and vomiting. Drugs known as growth factors are sometimes given to keep blood counts higher and reduce the chance of infection.

Other ways people can reduce the risk of infection are by avoiding exposure to germs as much as possible by carefully washing hands and not eating uncooked fruits and vegetables. Patients in treatment should also avoid large crowds and people who are sick. Fresh flowers and plants can carry mold, so they should be avoided as well.

During treatment, patients might also receive powerful antibiotics as added protection. They might start taking these at the first sign of infection, or even sooner as a preventive measure. If platelet counts are low, they might receive platelet transfusions. They might also get medicines or red blood cell transfusions if low red blood cell counts are causing shortness of breath or fatigue.

Monoclonal Antibodies

These substances can be made in the lab. They are like the antibodies made naturally by the body to fight infections. They can help the patient’s immune system to react and destroy cancer cells.

• Campath is the first of these antibodies approved to treat chronic lymphocytic leukemia. It is used in patients with CLL who are no longer being helped by chemotherapy. The major side effects are fever and chills. Campath can also lead to infections because it destroys many infection-fighting cells.
• Rituxan is an antibody used against certain kinds of lymphoma. It has also been useful in treating patients with CLL.

Surgery

Unlike with most other cancers, surgery is not generally used to treat leukemia. This is because leukemia is a disease of blood and bone marrow and it is not possible to bring about a cure with surgery.

Rarely, an operation may be done to remove the spleen. If leukemia spreads to the spleen it can cause the organ to become large enough to press on other organs and cause problems. If this happens, removing the spleen can provide relief, although it does not cure the leukemia. Another reason to remove the spleen is to improve blood cell counts. One of the spleen's normal functions is to remove worn-out blood cells from the bloodstream. If the spleen becomes too large, it may become too active in removing blood cells, leading to a shortage of red blood cells or platelets.

Radiation Therapy

Radiation therapy, the use of high energy x-rays to kill cancer cells, has a limited role to play in the treatment of people with leukemia. For some people with an enlarged organ such as the spleen, radiation might be used to shrink the swelling. It is also useful in treating bone pain caused by growth of leukemia cells within the bone marrow. The main short-term side effects of radiation treatment are sunburn-like changes in the treated area, tiredness, and a higher chance of infection.

Bone Marrow or Peripheral Blood Stem Cell Transplantation (SCT)

As noted earlier, chemotherapy can harm normal cells as well as cancer cells. A stem cell transplant offers a way for doctors to use the high doses of chemotherapy needed for effective treatment. Although the drugs destroy the patient's bone marrow, transplanted stem cells can restore the blood-producing bone marrow stem cells.

Stem cells for transplantation are collected from the bone marrow or from the bloodstream (in a process called apheresis). Bone marrow transplant was more common in the past. Now it has been largely replaced by cells taken from the bloodstream.

These blood-forming stem cells can come from either the patient or from a donor whose tissue type closely matches that of the patient. The donor may be a brother or sister or, less often, a person not related to the patient.

Stem cell transplants have been used in treating CLL. But often older patients do not do well following a donor transplant and may not survive.

The Transplant Process

• First, blood-forming stem cells are collected from either the patient or a matched donor.
• The cells are frozen and stored.
• Patients are then given very high doses of chemotherapy to kill the cancer cells.
• They may also receive total body radiation to kill any remaining cancer cells.
• After treatment, the stored stem cells are given to the patient as a blood transfusion.
• Then the waiting period begins as the stem cells settle in the patient's bone marrow and start to grow and produce blood cells.

People who receive a donor's stem cells are given drugs to prevent rejection as well as other medicines as needed to prevent infections. Usually around 10 to 21 days after the stem cells are infused they begin making new white blood cells. Then they begin making platelets, and finally, red blood cells.

Patients having SCT have to be kept away from germs (in protective isolation) as much as possible until their white blood cell count is at a safe level. They are kept in the hospital until the white cell count reaches a certain number, usually around 1,000. After they go home, they will be examined in the outpatient clinic almost every day for several weeks.

Stem cell transplantation is a complex treatment. If the doctors think that a person with leukemia might be helped by this treatment, it is important that it be done at a hospital where the staff have experience with the procedure. Some transplant programs may not have experience in certain transplants, especially those from unrelated donors. Stem cell transplantation is very expensive (more than $100,000) and requires a lengthy hospital stay. Because some insurance companies see it as an experimental treatment, they might not pay for it.

Side Effects of SCT

Side effects from a stem cell transplant can be divided into early and long-term effects. Early side effects are basically the same as those of any other type of high-dose chemotherapy (low blood cell counts, nausea, vomiting, hair loss, etc.). They are caused by damage to the bone marrow and other rapidly reproducing tissues of the body.

Side effects can go on for a long time. Sometimes they don't show up until years after the transplant. The long-term side effects could include the following:

• radiation damage to the lungs, causing shortness of breath
• graft-versus-host disease, which happens only in donor transplants
• damage to the ovaries causing infertility and loss of menstrual periods
• damage to the thyroid gland that causes problems with metabolism
• cataracts (damage to the lens of the eye)
• bone damage; if damage is severe, the patient will need to have part of the bone and joint replaced.
• Graft-versus-host disease is the main problem of a donor stem cell transplant. It happens when the immune system of the patient is taken over by that of the donor. The donor immune system then begins reacting against the patient’s other tissues and organs.

Symptoms can include severe skin rashes with itching and severe diarrhea. The liver and lungs may also be damaged. The patient may also become tired and have aching muscles. If severe enough, the disease can be fatal.

Doctors are also trying out a different way of doing the transplant that involves smaller doses of chemotherapy drugs. These "mini transplants" may make it possible for some patients over the age of 55 to have stem cell transplants.

Treatment of Chronic Lymphocytic Leukemia by Risk Group

Treatment options for people with CLL vary greatly depending on the stage of the disease and whether it is causing any symptoms. Perhaps the most important factor is the risk group. Although the staging system has been used in the past, testing for changes in the cells and chromosome may be more useful. Newer tests that will be very helpful are expected in the future.

Low-risk CLL: The outlook for this group is very good. Often no treatment is given right away. The doctor will keep close watch on the patient. Treatment can be started if it looks like the disease is getting worse or if the patient starts having symptoms. Treatment usually involves chemotherapy.

Intermediate and high-risk CLL: Patients in these risk groups who do not have symptoms may not need treatment right away. They can be observed to see if the disease gets worse or if they have new symptoms. People in these risk groups live for an average of 8 to 10 years. But some people who have very high-risk disease may best be treated with early stem cell transplantation.

In general, treatment would be chemotherapy. But doctors are beginning to study using monoclonal antibodies along with chemotherapy for CLL.

If the only problem is an enlarged spleen or swollen lymph nodes in one region of the body, treatment with lose-dose radiation is often used. Removing the spleen is another option if the enlarged spleen is causing problems.

If there are very high numbers of leukemia cells, the blood can be passed through a special machine that removes white blood cells and returns the rest of the blood to the patient. This process is called leukapheresis. This helps right away, but the effect is temporary.

Also, stem cell transplantation might be another option for certain patients, but its usefulness is not yet proven.

Treatment of Complications of CLL

CLL can cause serious problems with other parts of the blood. Rarely, it can change into a different, more aggressive, type of cancer called non-Hodgkin lymphoma. This change is known as Richter syndrome. If this happens, the patient will be treated for lymphoma. See our document on non-Hodgkin lymphoma for more information.

CLL can also change, though rarely, into the acute form of the disease--acute lymphocytic leukemia or ALL. If this happens, the patient will be treated with the chemotherapy used for ALL.

It’s also possible for the treatment of CLL to cause acute myeloid leukemia or AML, which is very aggressive and hard to treat.

Sometimes CLL changes a patient’s immune system in a way that causes it to attack his or her own red blood cells or blood platelets. These problems are treated with steroid drugs.

Treatment of Hairy Cell Leukemia (HCL)

People without symptoms do not have treatment. They have careful follow-up exams to watch for any changes or symptoms. Some people with HCL live for many years without any symptoms or treatment.

For those with low blood cell counts, doctors now recommend chemotherapy. Up to 80% to 90% of patients respond to chemotherapy, and the responses last more than 5 years in 75% of patients. If patients don’t fully respond to the chemo, doctors have found that giving monoclonal antibodies may completely get rid of the disease. Because this disease is so rare, too few people have been treated this way to be able to tell if it will work in the long run.

What Is Chronic Myeloid Leukemia?
 
Chronic myeloid leukemia (CML) is also known as chronic myelogenous leukemia. It is a type of cancer that starts in blood-forming cells of the bone marrow. It then invades the blood and can then spread to the lymph nodes, the spleen, liver, and other parts of the body. In contrast, other types of cancer can start in these organs and then spread to the bone marrow (or elsewhere). Those cancers are not leukemia. CML can also change into a fast-growing acute leukemia that invades almost any organ in the body.

How Many People Get Chronic Myeloid Leukemia?
 
The American Cancer Society estimates that in 2006 there will be about 4,500 new cases of chronic myeloid leukemia (CML) in the US. About 600 people in the United States will die of CML during 2006.

Chronic myelogenous leukemia affects mostly adults. The average age of people with CML is around 66 years. Only about 2% of CML patients are children. Because of dramatic improvements in treatment over the past few years, the survival rate of people newly diagnosed with CML is not known
 
What Causes Chronic Myeloid Leukemia? 
 
In general, there are very few known environmental risk factors for CML.

• Being exposed to high-dose radiation (from an atomic bomb blast or nuclear reactor accident)
• Sometimes a person inherits DNA changes from a parent that greatly increase the risk of getting certain types of cancer. But these inherited changes are very rarely the cause of CML. Changes linked to CML usually occur during life rather than having been inherited before birth.

Can Chronic Myeloid Leukemia Be Prevented?
 
Most people who develop CML do not have any risk factors. The cause of their leukemia remains unknown at this time. Because the cause is not known, there is no way to prevent most cases of CML.
 
How Is Chronic Myeloid Leukemia Found?
 
At this time, there are no special tests that can find chronic myeloid leukemia early. The best course of action is to report any symptoms to the doctor right away. These symptoms are discussed in the section that follows.

What are the Symptoms of Chronic Myeloid Leukemia?

Many people with CML have no symptoms at the time their cancer is found. In these cases, the cancer is often found by blood tests done for some other reason. Even when there are symptoms, they may be very general. Many of the symptoms of advanced CML happen because the leukemia cells replace the bone marrow’s blood-producing cells. As a result, people do not have enough blood cells and platelets that are working as they should.

• fatigue
• weakness
• weight loss
• fever
• bone pain
• Anemia is a result of a shortage of red blood cells. Anemia tiredness and, in more serious cases,  shortness of breath.
• Not having enough normal white blood cells increases the risk of infection. Although people with leukemia may have very high white blood cell counts, the cells are not normal and do not protect against infection very well.
• Not having enough blood platelets can lead to bruising, bleeding, frequent or severe nosebleeds, and bleeding from the gums. Some patients with CML have the opposite problem—too many platelets. But those platelets may not function as they should, leading to bleeding and bruising problems.
• Some patients have bone pain or joint pain caused by cancer cells spreading from the marrow to the surface of the bone or into the joint.
• Leukemia can also cause swelling of the liver and spleen. If these organs are enlarged, the person may notice fullness, even swelling, of the belly.  CML may sometimes spread to other organs.

Tests to Look for Leukemia

Blood cell counts and blood cell examination: Changes in the numbers of different blood cell types and how the cells look under a microscope can suggest leukemia. Most people with CML, for example, will have too many white blood cells, not enough red cells, and not enough platelets.

People with leukemia will have tests done to measure the amount of certain chemicals in the blood. These tests do not tell if they have leukemia but can help tell how well their kidneys and liver are working. The test results also help the doctor decide whether treatment is needed to correct low or high blood levels of certain minerals.

Bone marrow tests: In bone marrow aspiration, a thin needle is used to draw up a small amount of liquid bone marrow. The skin and the surface of the bone are first numbed, but the test can still be uncomfortable. During a bone marrow biopsy, a small cylinder of bone and marrow (about ½" long) is removed with a slightly larger needle.

Both samples are usually taken at the same time from the back of the hipbone. These tests are used to tell whether leukemia is present and also, if the person is having treatment, how well the disease is responding.

Lab Tests for CML

Any samples taken (blood, bone marrow, etc.) are looked at under a microscope by a doctor with special training (pathologist). The doctor looks at the size and shape of the cells as well as other features to classify the cells into specific types. An important goal of this process is to see whether the cells appear mature or not. The most immature cells are called blasts. These blasts are not effective in fighting infections, but they can reproduce, crowding out normal, mature cells. In addition, doctors may use 2 very precise lab tests to diagnose and classify leukemia: cytochemistry, and cytogenetics.

Imaging Studies

• Chest x-ray: This is a plain x-ray of the chest. It isn’t used to tell if someone has CML but rather to look for a lung infection.
• CT (computed tomography) scans: These are special kinds of x-rays in which a beam moves around the body, taking pictures from different angles. CT scans can show whether any organs are enlarged. CT scans take longer than regular x-rays. You will need to lie still on a table. The part of your body being scanned is placed within the scanner. The test is painless, but you will need to lie still for minutes at a time.
• MRI (magnetic resonance imaging): This test uses powerful magnets and radio waves to produce detailed, computer-generated pictures of the body. MRI scans are very helpful in looking at the brain and spinal cord. They take longer than CT scans, often up to an hour. You may need to lie inside a tube for the test.
• Ultrasound: In this test, sound waves are used to produce images of internal organs. Ultrasound can look for enlarged organs inside the abdomen. This is a very easy test to take. You simply lie on a table and a small wand (transducer) is moved over the part of the body being looked at.

How Is Chronic Myeloid Leukemia Staged?
 
Most types of cancer are assigned a stage based on the size of the tumor and how far it has spread. This system generally does not apply to leukemia because leukemia does not usually form a solid mass or tumor. Also, leukemia involves the bone marrow and, in many cases, has already spread to other organs in the body when it is found.

For CML, the outlook for the patient depends on information such as the subtype, features of the cells shown in lab tests and the results of imaging studies. This information helps guide treatment decisions.

CML is divided into 3 groups:

• Chronic phase: In this phase, blasts make up fewer than 10% of the cells in the blood or bone marrow. Patients usually have rather mild symptoms and respond to standard treatments.
• Accelerated phase: There are higher numbers of blasts in the blood or bone marrow in this phase. Patients in this phase often have fever, poor appetite, and weight loss. They do not respond to treatment as well as during the chronic phase.
• Blast phase (acute phase or blast crisis): At this point the chronic leukemia has changed into a very aggressive acute leukemia. There are even more blasts in the blood or bone marrow. Patients in this phase often have fever, poor appetite, and weight loss.

Other factors besides the stage can help predict a patient’s outlook for survival. These factors (called prognostic factors) are sometimes used along with staging to select treatment options. Factors that are linked to shorter survival time are called adverse prognostic factors. Those linked to longer survival are favorable prognostic factors.

Many of these factors are used in the Sokal system, which gives a score to predict outlook. It takes into account the person’s age, the percentage of blasts, the size of the spleen, the numbers of various kinds of cells, and other factors. Your doctor can tell you if any of these factors apply in your case and what they mean.

How Is Chronic Myeloid Leukemia Treated?

• Interferon

Interferons are substances naturally made by several types of cells. They can be used to slow the growth of leukemia cells and are injected under the skin daily.

Side effects include muscle aches, bone pain, headaches, problems with thinking, tiredness, nausea, and vomiting. These problems usually improve once treatment is over. But many patients will stop treatment because of these side effects.At one time Interferon was the main treatment for CML but a new drug, described below, is now used more often.

• Kinase Inhibitors

A drug called imatinib mesylate (Gleevec) has has changed the way doctors treat people in the early stages of CML. The drug works by slowing down or stopping cancer cells from making an abnormal enzyme (kinase) that allows them to grow. The drug is taken by mouth. Almost all patients respond to the drug and the side effects are much less than those from other treatments.

Possible side effects could include diarrhea, nausea, muscle pain, and tiredness—but these are generally mild. Some people get itchy skin rashes. Many people have fluid retention that can be helped with diuretics or “water pills.” Sometimes people taking the drug will have a drop in their red blood cell and platelet counts and the doctor may stop the drug for a short period.

Other drugs, over-the-counter as well as supplements, can interfere with this treatment. So if you are taking imatinib, be sure to check with your doctor before you start taking anything else.

• Chemotherapy

Chemotherapy refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they spread throughout the body. This treatment is often useful for cancers like leukemia that spread throughout the body.

For CML, though, chemotherapy is generally used only if other treatments have stopped working. If chemotherapy is used, it often includes a combination of several drugs given over a period of time.

While chemotherapy drugs kill cancer cells, they can also damage normal cells. This happens because they target rapidly growing cells such as cancer cells, but in the process they also damage other fast growing cells.

Bone marrow, the lining of the mouth and intestines, and hair follicles all grow rapidly and are likely to be damaged by chemotherapy. As a result, people being treated with chemotherapy may have a higher risk of infection (from low white blood cell counts), may bruise or bleed easily (from low blood platelets), and tire easily (from low red blood cell counts). Other side effects of chemotherapy can include short-term hair loss, nausea, vomiting, and loss of appetite.

These side effects usually go away shortly after treatment ends. There are often ways to manage these side effects during treatment. For example, there are drugs than can be taken along with the chemotherapy to prevent or reduce nausea and vomiting. Drugs known as growth factors are sometimes given to keep blood counts higher and reduce the chance of infection.

Other ways people can reduce the risk of infection are by avoiding exposure to germs as much as possible by carefully washing hands often and not eating uncooked fruits and vegetables. Patients in treatment should also avoid large crowds and people who are sick, and fresh flowers and plants because they carry mold.

During treatment, patients might also receive powerful antibiotics as added protection. They might start taking these at the first sign of infection, or even sooner as a preventive measure. If platelet counts are low, they might receive platelet transfusions. They might also get medicines or red blood cell transfusions if low red blood cell counts are causing shortness of breath or fatigue.

• Radiation Therapy

Radiation therapy, the use of high energy x-rays to kill cancer cells, has a limited role to play in the treatment of people with leukemia. For some people with an enlarged organ such as the spleen, radiation might be used to shrink the swelling. It is also useful in treating bone pain caused by growth of leukemia cells within the bone marrow. The main short-term side effects of radiation treatment are sunburn-like changes in the treated area, tiredness, and a higher chance of infection.

• Surgery

Unlike with most other cancers, surgery is not generally used to treat leukemia. This is because leukemia is a disease of blood and bone marrow and surgery cannot cure this type of cancer.

Rarely, an operation may be done to remove the spleen. If leukemia spreads to the spleen it can cause the organ to become large enough to press on other organs and cause problems. If this happens, removing the spleen can provide relief, although it does not cure the leukemia. Another reason to remove the spleen is to improve blood cell counts. One of the spleen's normal functions is to remove worn-out blood cells from the bloodstream. If the spleen becomes too large, it may become too active in removing blood cells, leading to a shortage of red blood cells or platelets.

• Bone Marrow or Peripheral Blood Stem Cell Transplantation (SCT)

As noted earlier, chemotherapy can harm normal cells as well as cancer cells. A blood-forming stem cell transplant offers a way for doctors to use the high doses of chemotherapy needed for effective treatment. Although the drugs destroy the patient's bone marrow, transplanted stem cells can restore the blood-producing bone marrow stem cells.

Blood-forming stem cells for transplantation are collected from the bone marrow or from the bloodstream in a process called apheresis. Bone marrow transplant was more common in the past. Now it has been largely replaced by cells taken from the bloodstream. The cells are frozen and stored.

These stem cells can come from either the patient or from a donor. For people with CML, a donor's cells are most often used because it's hard to find cancer-free cells in the patient's own marrow. Donor stem cell transplants are the major kind of transplant done for patients with CML. One problem is that they can only be used in young people. Patients over the age of 50-55 usually cannot tolerate them and will have a high chance of dying from the procedure.

Patients having a transplant are given very high doses of chemotherapy to kill the cancer cells. They may also receive total body radiation to kill any remaining cancer cells. After treatment, the stored stem cells are given to the patient as a blood transfusion. Then the waiting period begins as the stem cells settle in the patient's bone marrow and start to grow and produce blood cells.

People who receive a donor's stem cells are given drugs to prevent rejection as well as other medicines as needed to prevent infections. Usually around 10 to 21 days after the stem cells are infused they begin making new white blood cells. Then they begin making platelets, and finally, red blood cells.

Patients having SCT have to be kept away from germs (in protective isolation) as much as possible until their white blood cell count is at a safe level. They are kept in the hospital until the white cell count reaches a certain number, usually around 1,000. They may also receive platelet transfusions as an outpatient after they leave.

Stem cell transplantation is a complex treatment. If the doctors think that a person with leukemia might be helped by this treatment, it is important that it be done at a hospital where the staff has experience with the procedure. Some transplant programs may not have experience in certain transplants, especially those from unrelated donors.

Stem cell transplantation is very expensive (usually more than $100,000) and requires a lengthy hospital stay. Because some insurance companies see it as an experimental treatment, they might not pay for it.

Lastly, the use of imatinib has changed the way doctors look at SCT. In the past, this disease was almost always fatal. This led doctors to recommend transplants for most patients younger than 50 or 55. Now doctors are often taking a wait and see approach, because they aren’t sure who will need a transplant or whether patients who respond well to Gleevec will eventually be cured.

Side Effects of SCT

Side effects from a stem cell transplant can be divided into early and long-term effects. Early side effects are basically the same as those of any other type of high-dose chemotherapy (low blood cell counts, nausea, vomiting, hair loss, etc.). They are caused by damage to the bone marrow and other rapidly reproducing tissues of the body.

Side effects can go on for a long time. Sometimes they don't show up until years after the transplant. The long-term side effects could include the following:

• radiation damage to the lungs, causing shortness of breath
• graft-versus-host disease, which happens only in donor transplants.
• damage to the ovaries causing infertility and loss of menstrual periods
• damage to the thyroid gland that causes problems with metabolism
• cataracts (damage to the lens of the eye)
• bone damage; if damage is severe, the patient will need to have part of the bone and joint replaced.
• Graft-versus-host disease is the main problem of a donor stem cell transplant. It happens when the immune system of the patient is taken over by that of the donor. The donor immune system then begins reacting against the patient’s other tissues and organs.

Symptoms can include severe skin rashes with itching and severe diarrhea. The liver and lungs may also be damaged. The patient may also become tired and have aching muscles. If severe enough, the disease can be fatal.

Doctors are also trying out a different way of doing the transplant that involves smaller doses of chemotherapy drugs. These “mini transplants” may make it possible for some older patients to have stem cell transplants.

Treatment of CML by Phase

Treatment choices for people with CML depend on the phase of the disease, their age, other prognostic factors, and whether there is a potential stem cell donor with a matching tissue type.

Chronic phase: The drug imatinib (Gleevec) has completely changed the treatment of CML. With this pill, 9 out of 10 patients will have a total remission of their disease. Most of these patients are still in remission after 4 years. No one yet knows how long these remissions will last and whether the new drug will actually cure people with CML. Some patients, however, stop responding to the drug. Sometimes this can be overcome by increasing the dose of imatinib. Also, new drugs, more powerful than imatinib have been developed.

No one yet knows how long these remissions will last and whether imatinib will actually cure patients with CML. For patients with a good response, one estimate is that survival time could be around 16 years.

Until now, high-dose chemotherapy with total body radiation, followed by stem cell transplant has been the only known way to cure this disease. But the side effects of SCT are serious and can be fatal. Stem cell transplant is not recommended for older patients. For older patients, a “mini transplant” may be an option.

Because no one knows the long term results for imatinib, young people who might be good candidates for stem cell transplant might be reluctant to have the procedure. Doctors will want to watch patients under 30-35 years of age very closely. If they do not go into and remain in complete remission on the drug, SCT should be recommended. For older patients, a wait-and-see approach may be safer.

CML can become resistant to the drug, although this seems to be rare in the chronic phase.

Accelerated phase: Treatment choices in this phase are much like those used in the chronic phase. Although imatinib has brought about remissions for people in this phase, these have not lasted very long. For the most part, patients in this phase are less likely to have a long remission with any treatment. About 2 out of 10 patients have some response to chemotherapy, but their remission is usually shorter than 6 months. For those patients, there are some newer drugs that, seem to work better in this phase.

About 15% of patients in this phase are alive several years after stem cell transplantation. This may be the best option for younger patients. Most doctors prefer that the leukemia be in remission before starting the transplant procedure. To achieve this, chemotherapy will often be used.

Blast phase: In this phase of CML, the cells look like cells of acute myeloid leukemia (AML). But they are resistant to the drugs that are used to treat AML. Sometimes standard treatment for AML will bring about a short remission. If that happens, then some sort of transplant might be done.

A small number of patients in this phase have blast cells that look like those of acute lymphocytic leukemia (ALL). These cells respond better to chemotherapy and a remission may be achieved.

Stem cell transplant from a donor rarely works for blast phase CML. Treatment to relieve symptoms (palliative treatment) is an important part of care for patients in this phase. Radiation treatment can help bone pain, and chemotherapy may relieve some symptoms for a few months. The effects of CML that has spread to the nervous system can be relieved in many cases by chemotherapy or by radiation to the brain.

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