Leukemia - Acute Lymphocytic (ALL)

What Is Acute Lymphocytic Leukemia?

Leukemia is a type of cancer that starts in the soft, inner part of the bones (bone marrow) and 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. 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. Both children and adults can get leukemia.
The term "acute" means that the cancer develops quickly, and if not treated, could be fatal in a few months. "Lymphocytic" (limf-o-sit-ik) means that the cancer starts from cells called lymphocytes (limf-o-sites). Acute lymphocytic leukemia (ALL) is a type of cancer that starts from these white blood cells in the bone marrow. ALL is sometimes called acute lymphoblastic leukemia.
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 of leukemia and other factors.
Normal Bone Marrow, Blood, and Lymphoid Tissue
In order to understand the different types of leukemia, it helps to have some basic knowledge of the blood and lymph systems.
Bone Marrow
Bone marrow is the soft, spongy, inner part of bones. All of the different types of blood cells are made in the bone marrow. Bone marrow is made up of blood stem cells, blood-forming cells, fat cells, and tissues that aid the growth of blood cells.
The blood-forming cells come from blood stem cells. They only make new blood-forming cells and not other kinds of cells. (They are different from embryonic stem cells which are formed from a developing fetus and can grow to become other kinds of cells in the body.) The blood-forming cells can develop into 1 of the 3 main types of blood cell: red blood cells, white blood cells, or platelets.
Red Blood Cells
Red blood cells carry oxygen from the lungs to all other tissues of the body. They also carry away carbon dioxide, a waste product of cell activity. A shortage of red blood cells causes weakness, shortness of breath, and tiredness.
Platelets
Platelets are actually pieces that break off from certain bone marrow cells.. Platelets help stop bleeding by plugging up holes in blood vessels caused by cuts or bruises. A shortage of platelets can cause a person to bleed or bruise easily.
White Blood Cells
White blood cells help defend the body against germs such as viruses and bacteria. There are quite a few types of white blood cells. Each has a special role to play in protecting the body against infection. The 3 main types of white blood cells are granulocytes, monocytes, and lymphocytes.
The immune system is made up mainly of lymphoid tissue (also known as lymphatic tissue). The main cell type that forms lymphoid tissue is the lymphocyte, a kind of white blood cell. The two main types of lymphocytes are called B-cells and T-cells. Normal T-cells and B-cells do different jobs within the immune system.
Any of the blood-forming cells can turn into a leukemic cell. Once that happens, the cell can reproduce to form many new cancer cells. These cells can overwhelm the bone marrow, spill out into the bloodstream, and spread to other organs. Acute lymphocytic leukemia starts from early forms of the lymphocytes.
Types of Leukemia
Not all leukemias are the same. Leukemias are divided into 4 main types. Knowing the exact type of leukemia can help doctors better predict each patient's outlook (prognosis) and select the best treatment.
The major types of leukemia are based on these factors:
acute or chronic
lymphocytic or myeloid
Acute Leukemia Versus Chronic Leukemia
The first factor to take into account to classify a patient's leukemia is whether most of the abnormal cells look like normal mature white blood cells or whether they look more like stem cells (have not matured.)
Acute leukemia: In acute leukemia, the bone marrow cells don’t mature properly. These immature cells build up and crowd out normal cells. Without treatment, most patients with acute leukemia would live only a few months. Some types of acute leukemia respond well to treatment and many patients are cured. People with other types often do not do as well.
Chronic leukemia: In chronic leukemia the cells look mature, but they are not really normal and they can’t fight infection they way they should. They also live too long, so that they build up and crowd out normal bone marrow cells. Chronic leukemias tend to progress over a longer period of time, and most patients can live for many years. However, chronic leukemias are generally harder to cure than acute leukemias.
Myeloid Leukemia Versus Lymphocytic Leukemia
The second factor to take into account is the type of bone marrow cells that are involved.
Myeloid leukemia: Myeloid leukemia mainly develops from white blood cells such as granulocytes or monocytes, but can also form from the cells that mature into platelets or red blood cells.
Lymphocytic leukemia: Lymphocytic leukemias develop from lymphocytes (a type of white blood cell) in the bone marrow.
Most cases of leukemia can be sorted into 1 of the 4 main types shown in the table below.
Acute lymphocytic leukemia (ALL)
Acute myeloid leukemia (AML)
Chronic lymphocytic leukemia (CLL)
Chronic myeloid leukemia (CML)
Although ALL is the most common of the 4 major types of leukemia among children, it is the least common type among adults.

How Is Acute Lymphocytic Leukemia Found?

Signs and Symptoms of Acute Lymphocytic Leukemia
At this time, there are no special tests that can find acute leukemia early. The best course of action is to report any symptoms (described below) to the doctor right away. Keep in mind that these symptoms are most often caused by something other than cancer.
General Symptoms
General symptoms of ALL can include weight loss, fever, and loss of appetite. Of course, AlL is not the only problem that causes these symptoms. They are most often caused by something other than cancer.
Shortage of Blood Cells
Most symptoms of ALL are caused by a shortage of normal blood cells, which happens when the leukemia cells crowd normal blood-making cells in the bone marrow. As a result, the person doesn't have enough normal red blood cells, white blood cells, and platelets. These shortages show up on blood tests, but they can also cause symptoms.
Anemia is a shortage of red blood cells. It can cause tiredness, weakness, feeling dizzy, cold, or lightheaded, and shortness of breath.
A shortage of normal white blood cells increases the risk of infections. Although peole with leukemia may have very high white blood cell counts, the cells are usually not normal and can not protect against infection. Fevers and other signs of infection are common symptoms of ALL.
A shortage of blood platelets can lead to excess bruising, bleeding, frequent or severe nosebleeds, and bleeding gums.
Bone or Joint Pain
Some patients have bone pain or joint pain caused by the buildup of leukemia cells in bones or joints.
Swelling In The Belly
Leukemia cells may collect in the liver and spleen, causing them to enlarge. This may be noticed as a fullness or swelling of the belly.
Enlarged Lymph Nodes
ALL may spread to lymph nodes. If the nodes are close to the surface of the body, they may be noticed as lumps under the skin. Lymph nodes inside the chest or abdomen may also swell, but these can be found only by imaging tests such as CT or MRI scans.
Spread To Other Organs
Less often, 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, and blurred vision. ALL may also spread to the chest, where it can cause fluid buildup and trouble breathing.
Enlarged Thymus Gland
One type of ALL (the T-cell subtype) often affects the thymus, which is a small gland in the middle of the chest found behind the breastbone and in front of the windpipe. An enlarged thymus can press on the 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 the thymus presses on the SVC, it can cause the head and arms to swell (SVC syndrome). This can affect the brain and is life threatening. People with SVC syndrome need treatment right away.

How Is Acute Lymphocytic Leukemia Treated?

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 ALL. This is followed by a discussion of the typical treatment approach for ALL.
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
Chemotherapy (often referred to as simply, "chemo") is the major treatment for ALL. Surgery and radiation may be used in some cases.
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. 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 help 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 may carry germs.
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 during treatment. When these cells die, they release contents into the bloodstream that can affect the kidneys, heart, and nervous system. Extra fluids or certain drugs that help rid the body of these substances 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. 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) at a later time. Less often, people cured of leukemia may later develop 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 chemotherapy.
Targeted Therapy
Newer drugs such as imatinib (Gleevec) have been used to successfully treat chronic myeloid leukemia (CML). These drugs target certain parts of cancer cells. Clinical trials are going on now to see if these drugs 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. A common side effect is swelling around the eyes or in the hands or feet. Other possible side effects include diarrhea, nausea, muscle pain, fatigue and skin rashes, as well as lower red blood cell and platelet counts at the start of treatment. These side effects are often mild.
Surgery
Surgery is not generally used to treat leukemia because it 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. This lowers the number of needle sticks needed during treatment. The patient must 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. It might also be used to reduce pain when the leukemia has spread to a bone if chemo hasn't helped
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 chemo is used instead.
Bone Marrow or Peripheral Blood Stem Cell Transplant
While very high doses of chemotherapy drugs might work better to kill cancer cells, they can cause severe damage to bone marrow cells which could be fatal.. Stem cell transplantats (SCT) offer 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.
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 has largely been replaced by peripheral blood stem cell transplant (PBSCT). 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.
The Transplant Process
The treatment works like this: stem cells for are collected from the bloodstream in a process called apheresis. The 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. 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 receive a donor's stem cells are given drugs to prevent rejection as well as other medicines as 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 is very expensive (more than $100,000) and involves 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 on a transplant.
Side Effects of Stem Cell Transplant
Side effects from stem cell transplant 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. 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
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 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 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. Some may be able to have what is called a "mini-transplant" (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 under way to find out how useful it may be against ALL. To learn more about stem cell transplants, see the American Cancer Society document, Bone Marrow & Peripheral Blood Stem Cell Transplants.
Treatment of ALL
For ALL, chemotherapy 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.
Induction
The purpose of the first phase, sometimes called remission induction, is to bring about a remission. A remission is the disappearance of the signs and symptoms of the cancer. 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 often consists of fairly short course of chemotherapy using many of the same drugs that were used before. This treatment phase lasts from one to a few months. Central nervous system treatment may be continued at this time. Doctors may suggest a stem cell transplant at this time 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 consists of lower doses of chemotherapy drugs, lasts about 2 years. Central nervous system treatment may also be continued.
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.
Response Rates
In general, about 70% to 90% of patients will have a complete response to these treatments. That means that leukemia can no longer be seen in their bone marrow (remission). But about half of these patients will relapse (the leukemia will come back), 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 considered.
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. In these cases, more chemotherapy 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, 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.
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 the 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 more mild chemotherapy to slow the growth of the leukemia 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, the patient might want to think about hospice care. Most of the time, this can be given at home. Hospice focuses on the patient's 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 one can at this difficult stage.
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.