This information represents the views of the doctors and nurses serving on the American Cancer Society's Cancer Information Database Editorial Board. These views are based on their interpretation of studies published in medical journals, as well as their own professional experience.

The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your family make informed decisions, together with your doctor.

Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. Don't hesitate to ask him or her questions about your treatment options.

This section starts with general comments about types of treatments used for acute lymphocytic leukemia (ALL). After this you will find a review of the typical treatment plan for ALL in adults.

As noted before, ALL is not one 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 (often called "chemo") is the major treatment for ALL. Surgery and radiation may be used in some cases.

Chemotherapy

Chemo 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. Chemo for ALL involves the use of several drugs given over a long period of time (often about 2 years).

Doctors give chemo in cycles. A round of treatment is followed by a rest period to allow the body time to recover.

Side effects of chemo

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

The side effects of chemo 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 (from low white blood cells)
• easy bruising or bleeding (from low blood platelets)
• tiredness (from 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 that can be taken along with the chemo to help prevent or reduce nausea and vomiting. Drugs called growth factors are sometimes given to keep blood counts higher and reduce the chance of infection.

If your white blood cell counts are very low during treatment, you can help reduce your risk of infection by avoiding germs. During this time, your doctor or nurse may tell you to:

• Wash your hands often.
• Avoid fresh, uncooked fruits and vegetables and other foods that might carry germs.
• Avoid fresh flowers and plants because they may carry mold.
• Make sure other people wash their hands when they come in contact with you.
• Avoid large crowds and people who are sick (wearing a surgical mask can help protect you).

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

Tumor lysis syndrome is a side effect caused by the rapid breakdown of leukemia cells during treatment. When these cells die, they break open and release their contents into the bloodstream. This cell waste can affect the kidneys, heart, and nervous system. Extra fluids or certain drugs can help the body get rid of these substances.

Organs that could be damaged by chemo 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, the drugs may have to be reduced or stopped. Be sure to tell your doctor about any problems you have.

One of the most serious side effects of ALL treatment is the increased risk of getting acute myelogenous leukemia (AML) later. Less often, people cured of leukemia may later get non-Hodgkin lymphoma or other cancers. Of course, the risk of getting these second cancers must be balanced against the clear benefit of treating a life-threatening disease such as leukemia with chemo.

Targeted therapy

In recent years, new drugs that target certain parts of cancer cells have been developed. These targeted therapies work differently than standard chemo drugs. They often have less severe side effects. Drugs like imatinib (Gleevec^®) have been used to successfully treat chronic myeloid leukemia (CML). Studies are going on now to see whether these drugs will be helpful in treating some people with ALL. Early reports have shown they may help more patients get the ALL under control (in remission) after treatment and may help keep the leukemia from coming back. But larger studies are needed to confirm this. A common side effect is swelling around the eyes or in the hands or feet. Other possible side effects include diarrhea, nausea, muscle pain, extreme tiredness (fatigue), and skin rashes, as well as lower red blood cell and platelet counts at the start of treatment.

Surgery

Surgery has a very small role in the treatment of ALL. Because leukemia cells spread widely throughout the bone marrow and to many other organs, it is not possible to cure this type of cancer by surgery. But surgery may be used to help deliver treatment. For example, a plastic tube can be placed into a large vein. The tube, called a venous access device, allows drugs to be given and blood samples removed. This lowers the number of needle sticks needed during treatment. The patient must learn how to take care of the venous access 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. It might also be used to reduce pain when the leukemia has spread to a bone if chemo hasn't helped

Radiation to the whole 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 tumor if it is pressing on the windpipe. But more often chemo is used instead.

The possible side effects of radiation depend on where it is aimed. There may be sunburn-like skin changes in the treated area. Radiation to the belly (abdomen) can sometimes cause nausea, vomiting, or diarrhea. For radiation that includes large parts of the body, the effects may include extreme tiredness (called fatigue) and an increased risk of infection.

Bone marrow or peripheral blood stem cell transplant

While very high doses of chemo drugs might work better to kill ALL cells, they can cause severe damage to bone marrow cells which could be life-threatening. Stem cell transplants (SCT) offer a way for doctors to use high doses of chemo. Although the drugs destroy the patient's bone marrow, transplanted stem cells can restore the bone marrow's ability to make blood.

Stem cells for a transplant come from either from the blood or from the bone marrow. Bone marrow transplants were more common in the past, but they have largely been replaced by peripheral blood stem cell transplant (PBSCT).

Types of transplants

The stem cells can come from either the patient or from a matched donor. There is a good reason to use stem cells from someone else for the transplant. These cells seem to help fight any remaining leukemia cells through an immune reaction. This is called a "graft-versus-leukemia" reaction.

Allogeneic stem cell transplant: In an allogeneic transplant, the stem cells come from someone else -- usually a donor whose tissue type is a very close match to the patient's. The donor may be a brother or sister if they are a good match. Less often, an unrelated donor may be found. An allogeneic transplant is the preferred type of transplant for ALL when it is available.

Autologous stem cell transplant: In an autologous transplant, a patient's own stem cells are removed from his or her bone marrow or blood. They are frozen and stored while the person gets treatment (high-dose chemo and/or radiation). The stem cells are then given back to the patient after treatment.

One problem with autologous transplants is that it is hard to separate normal stem cells from leukemia cells in the bone marrow or blood samples. Even after treating the stem cells in the lab to try to kill or remove any leukemia cells, there is the risk of returning some leukemia cells with the stem cell transplant.

The transplant process

The transplant process works like this: stem cells are collected from the bloodstream in a process called apheresis. The cells are frozen and stored. Patients are then given very high doses of chemo to kill the cancer cells. The patient also gets total body radiation to kill any cancer cells that the chemo might miss. After treatment, the stored stem cells are given to the patient as a blood transfusion. The stem cells settle into the patient's bone marrow over the next several days and start to grow and make new blood cells.

People who get a donor's stem cells are given drugs to prevent rejection as well as other medicines if needed to prevent infections. Usually within a couple of weeks 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.

Some things to keep in mind

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 transplant costs a lot, often more than $100,000. It may involve a long hospital stay. Because some insurance companies see it as an experimental treatment, they might not pay for it. It is important to find out what your insurer will cover and what you might have to pay before deciding to have a transplant.

Side effects of stem cell transplant

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

Early side effects: Early side effects are much the same as those caused by any other type of high-dose chemo, such as nausea, vomiting, loss of appetite, mouth sores, and hair loss. One of the most common and serious short-term effects is the greater risk of infection. Antibiotics are often given to try to prevent these infections. Other side effects, like low red blood cell and platelet counts, may mean the patient will need transfusions.

Long-term side effects: Some side effects can last for a long time, or may not happen until years after the transplant. These long-term side effects can include the following:

• graft-versus-host disease (GVHD), which occurs only in a donor transplant (see below)
• radiation damage to the lungs, causing shortness of breath
• 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
• damage to the eye that can affect vision (cataracts)
• bone damage (If damage is severe, the patient may 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 bad enough, the disease can be fatal. Drugs that weaken 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.

"Mini transplant"

Most patients over the age of 55 can't have a regular transplant that uses high doses of chemo. But some may be able to have what is called a "mini-transplant" (also called a non-myeloablative transplant or reduced-intensity transplant), where they get lower doses of chemo and radiation that do not destroy the all cells in their bone marrow. They then are given the donor stem cells. These cells enter the body and form a new immune system which sees the leukemia cells as foreign and attacks them (a "graft-versus-leukemia" effect). This approach is still considered experimental, and studies are being done to find out how useful it may be against ALL.

To learn more about stem cell transplants, see our document, Bone Marrow & Peripheral Blood Stem Cell Transplants.

Treatment of ALL

For ALL, chemo treatments are given in the phases described below. The total treatment usually takes about 2 years, with the maintenance phase taking up most of this time. Treatment may be more or less intense, depending on the subtype of ALL and other prognostic factors.

Remission induction: The purpose of the first phase is to bring about a remission. A remission means there are no signs and symptoms of the cancer. More than one chemo drug will be used and high doses will be given. Treatment to keep the leukemia cells from spreading to the central nervous system is often started at this time (see below).

Consolidation: If the patient goes into remission, the next phase is often a fairly short course of chemo using many of the same drugs and high doses that were used before. This treatment phase lasts for a few months. Central nervous system treatment may be continued at this time. Doctors may suggest a stem cell transplant for patients who are at a high risk of relapse.

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 means lower doses of chemo drugs, lasts about 2 years. Central nervous system treatment may also be continued.

Central nervous system treatment: Because ALL often spreads to the brain and spinal cord, patients often get chemo put right into the spinal fluid or radiation therapy of the head to prevent this kind of spread.

Response rates to treatment

As a rule, about 80% to 90% of adult patients will have a complete response to these treatments. That means that leukemia can no longer be seen in their bone marrow (remission). But in about half of these patients the leukemia will come back (relapse), so the overall cure rate is around 30% to 40%.

What if the leukemia doesn't respond or comes back after treatment?

If the leukemia doesn't go away with the first treatment then newer or stronger doses of drugs may be tried, although they are less likely to work. A stem cell transplant may be tried if the leukemia can be put into at least partial remission. Clinical trials of new treatment approaches may also be an option.

If the leukemia comes back (recurs) 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. In these cases, more chemo might put the disease into remission, although this it is not likely to last. If a second remission can be achieved, most doctors will advise some type of stem cell transplant if possible.

If the leukemia keeps coming back or doesn't go away, over time the chemo will not be very helpful. If a stem cell transplant is not an option, a clinical trial (see section on "Clinical trials") might considered.

Some people want to keep on having treatment to fight the leukemia as long as they can. It is a good idea, though, to think about the odds of more treatment doing any good before making the decision to continue. Some people are tempted to try more chemo, for example, even when their doctors say that the odds of benefit are less than 1%. In these cases, it is important to think about and understand your reasons for choosing this plan.

Palliative treatment

If a clinical trial is not an option, then it may be time to focus on relieving symptoms rather than curing the cancer. This is known as palliative treatment. The doctor may suggest milder chemo to try to slow the growth of the leukemia in order to reduce symptoms.

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. Nausea and loss of appetite may be helped by high-calorie food supplements and medicines. Antibiotics may be needed to treat infection.

At some point, you might want to think about hospice care. Most of the time, this can be given at home. Hospice focuses on your comfort by taking care of any symptoms or other problems. It means that the goal of care is on living life as fully as possible and feeling as well as possible.

While the hope for a cure may not be as bright, there is still hope for good times with family and friends -- times that can bring happiness and meaning. In a way, pausing at this time in your cancer treatment offers a chance to refocus on the most important things in your life. This is the time to do some things you've always wanted to do and to stop doing the things you no longer want to do.

Last Medical Review: 08/07/2009
Last Revised: 08/07/2009