Acute lymphocytic leukemia (ALL) is a type of cancer of the blood and bone marrow — the spongy tissue inside bones where blood cells are made.

The word "acute" in acute lymphocytic leukemia comes from the fact that the disease progresses rapidly and affects immature blood cells, rather than mature ones. The "lymphocytic" in acute lymphocytic leukemia refers to the white blood cells called lymphocytes, which ALL affects. Acute lymphocytic leukemia is also known as acute lymphoblastic leukemia and acute childhood leukemia.

Acute lymphocytic leukemia is the most common type of cancer in children, and treatments result in a good chance for a cure. Acute lymphocytic leukemia can also occur in adults, though the prognosis is not as optimistic.

Signs and symptoms of acute lymphocytic leukemia may include:

• Bleeding from the gums
• Fever
• Frequent infections
• Frequent or severe nosebleeds
• Loss of appetite
• Lumps caused by swollen lymph nodes in and around the neck, underarm, stomach or groin
• Pale skin
• Shortness of breath
• Weight loss
• Weakness, fatigue or a general decrease in energy

When to see a doctor
Make an appointment with your doctor or your child's doctor if you notice any persistent signs and symptoms that concern you. Many signs and symptoms of acute lymphocytic leukemia mimic those of the flu. However, flu signs and symptoms will eventually improve. If signs and symptoms don't improve as expected, make an appointment with your doctor.

Acute lymphocytic leukemia occurs when a bone marrow cell develops errors in its DNA. The errors tell the cell to continue growing and dividing, when a healthy cell would normally die. When this happens, blood cell production goes awry. The bone marrow produces immature cells that develop into leukemic white blood cells called lymphoblasts. These abnormal cells are unable to function properly, and they can build up and crowd out healthy cells.

It's not clear what causes the DNA mutations that can lead to acute lymphocytic leukemia. But doctors have found that most cases of acute lymphocytic leukemia aren't inherited.

Factors that may increase the risk of acute lymphocytic leukemia include:

• Cancer therapy. Children and adults who've had certain types of chemotherapy and radiation therapy or treatment for other kinds of cancer may have a small, but greater, risk of developing acute lymphocytic leukemia than does the general population.
• Exposure to radiation. People exposed to very high levels of radiation, such as survivors of an atomic bomb blast or a nuclear reactor accident, have an increased risk of developing acute lymphocytic leukemia. This includes children who were exposed to significant radiation during the first months of prenatal development.
• Genetic disorders. Certain genetic disorders, such as Down syndrome, are associated with some increased risk of acute lymphocytic leukemia.
• Having a brother or sister with ALL. People who have a sibling, including a twin, with acute lymphocytic leukemia are slightly more likely to develop ALL.

However, most people with acute lymphocytic leukemia have no known risk factors.

You're likely to start by first seeing your family doctor or a general practitioner. However, in some cases when you call to set up an appointment, you may be referred immediately to a doctor who specializes in treating diseases and conditions of the blood and bone marrow (hematologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from the doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance.
• Write down any symptoms you or your child is experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes in your life or your child's life.
• Make a list of all medications, as well as any vitamins or supplements, that you or your child is taking.
• Take a family member or friend along, if possible. Sometimes it can be difficult to absorb all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Time with the doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For acute lymphocytic leukemia, some basic questions to ask the doctor include:

• What is likely causing these symptoms?
• What are other possible causes for these symptoms?
• What kinds of tests are necessary?
• Is this condition likely temporary or chronic?
• What is the best course of action?
• What are the alternatives to the primary approach that you're suggesting?
• How can existing health conditions be best managed with ALL?
• Are there any restrictions that need to be followed?
• Is it necessary to see a specialist? What will that cost, and will my insurance cover it?
• Is there a generic alternative to the medicine you're prescribing?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend?
• What will determine whether I should plan for a follow-up visit?

In addition to the questions that you've prepared to ask the doctor, don't hesitate to ask questions during the appointment at any time that you don't understand something.

What to expect from the doctor
The doctor is likely to ask you a number of questions. Being ready to answer them may allow time to cover other points you want to address. Your doctor may ask:

• When did symptoms first begin?
• Have these symptoms been continuous, or occasional?
• How severe are these symptoms?
• What, if anything, seems to improve these symptoms?
• What, if anything, appears to worsen these symptoms?

What you can do in the meantime
Avoid activity that seems to worsen any signs and symptoms. For instance, if you or your child is feeling fatigued, allow for more rest. Determine which of the day's activities are most important, and focus on accomplishing those tasks.

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Bone marrow test

Tests and procedures used to diagnose acute lymphocytic leukemia include:

• Blood tests. A blood test may reveal too many white blood cells, not enough red blood cells and not enough platelets. A blood test may also show the presence of blast cells — immature cells normally found in the bone marrow but not circulating in the blood.
• Bone marrow test. During bone marrow aspiration, a needle is used to remove a sample of bone marrow from the hipbone to look for leukemia cells. The sample is sent to a lab for testing. Doctors in the lab will classify blood cells into specific types based on their size, shape and other features. They also look for certain changes in the cancer cells and find out whether the leukemia cells began from the B lymphocytes or T lymphocytes. This information helps your doctor develop a treatment plan.
• Imaging tests. Imaging tests such as a chest X-ray, computerized tomography (CT) scan or an ultrasound examination may help determine whether cancer has spread to the brain and spinal cord or other parts of the body.
• Spinal fluid test. A lumbar puncture test, also called a spinal tap, may be used to collect a sample of spinal fluid. The sample is tested to see whether cancer cells have spread to the spinal fluid.

Determining a risk group in children with ALL
Doctors use several factors to determine a risk group for children with acute lymphocytic leukemia. The risk refers to the chance that cancer will recur after treatment. Risk groups include standard, high-risk or very high-risk categories. Your child's risk group helps determine his or her treatment. Factors used to determine risk group include:

• Age. Infants and children older than 10 are generally placed in a high-risk category.
• White blood cell count. The higher the white blood cell count at the time of diagnosis, the higher the risk group.
• Where cancer began. Immunophenotype testing determines whether the cancer began in the B lymphocytes (B-cell ALL) or the T lymphocytes (T-cell ALL). About 85 percent of cases are B cell, and about 15 percent are T cell. In general, children with B-cell ALL do better than those with T-cell ALL. So children with B-cell ALL are usually in a lower risk group, while those with T-cell ALL are in a higher risk group.
• Specific changes within the cancer cells. Cytogenic testing looks for changes to the chromosomes in the lymphocytes. The presence of certain kinds of hard-to-treat mutations also places children in a higher risk group.

In general, treatment for acute lymphocytic leukemia falls into separate phases:

• Induction therapy. The purpose of the first phase of treatment is to kill most of the leukemia cells in the blood and bone marrow.
• Consolidation therapy. Also called post-remission therapy, this phase of treatment is aimed at destroying the leukemia cells remaining in the brain or spinal cord.
• Maintenance therapy. The third phase of treatment prevents leukemia cells from regrowing. The treatments used in this stage are often given at much lower doses.
• Preventive treatment to the spinal cord. People with acute lymphocytic leukemia may also receive treatment to kill leukemia cells hiding in the central nervous system during each phase of therapy. In this type of treatment, chemotherapy drugs are injected directly into the fluid that covers the spinal cord. This kills cancer cells that can't be reached by chemotherapy drugs given by mouth or through an intravenous line.

The phases of treatment typically take two and a half to three and a half years. Treatments may include:

• Chemotherapy. Chemotherapy, which uses drugs to kill cancer cells, is the most common form of induction therapy for children and adults with acute lymphocytic leukemia. Chemotherapy induction therapy typically requires an extended hospital stay because the drugs destroy many normal blood cells in the process of killing leukemia cells. This can cause anemia, infection and bleeding.

  Chemotherapy drugs can also be used in the consolidation and maintenance phases, but these phases are usually less intensive regimens and don't require staying in the hospital.

• Targeted drug therapy. Targeted drugs attack specific abnormalities present in cancer cells that help them grow and thrive. One targeted drug, imatinib (Gleevec), specifically attacks cancer cells that have a certain abnormality called the Philadelphia chromosome. The drug dasatinib (Sprycel) works in a similar way. These drugs are only approved for people with the Philadelphia chromosome-positive form of ALL. Another drug, rituximab (Rituxan), targets cancer cells that have an overabundance of a certain protein. Targeted drug treatments may be combined with chemotherapy drugs.
• Radiation therapy. Radiation therapy uses high-powered beams, such as X-rays, to kill cancer cells. If the cancer cells have spread to the central nervous system, your doctor may recommend radiation therapy.

• Bone marrow stem cell transplant. A bone marrow stem cell transplant may be used as consolidation therapy in people at high risk of relapse or for treating relapse when it occurs. This procedure allows someone with leukemia to re-establish healthy stem cells by replacing leukemic bone marrow with leukemia-free marrow.

  A bone marrow stem cell transplant begins with high doses of chemotherapy or radiation to destroy any leukemia-producing bone marrow. The marrow is then replaced by bone marrow from a compatible donor (allogeneic transplant). In some cases, people are able to use their own bone marrow for transplantation (autologous transplant). This may be possible if you or your child goes into remission and healthy bone marrow is then harvested for a future transplant.

• Clinical trials. Clinical trials are experiments to test new cancer treatments and new ways of using existing treatments. While clinical trials give you or your child a chance to try the latest cancer treatment, they can't guarantee a cure. Discuss the benefits and risks of clinical trials with your doctor.

No alternative treatments have been found to cure acute lymphocytic leukemia. But some alternative therapies may help ease the side effects of cancer treatment and make you or your child more comfortable.

Alternative treatments that may ease signs and symptoms include:

• Acupuncture
• Aromatherapy
• Massage
• Meditation
• Relaxation exercises

Talk to your doctor if you're interested in trying alternative treatments. Some alternative therapies are safe, while others can interfere with your cancer treatments.

Survival rates for acute lymphocytic leukemia have improved dramatically during the past 30 years. But encouraging survival rates don't always seem that encouraging at the moment you learn that you or your child has leukemia.

Additionally, although treatment for adult and childhood acute lymphocytic leukemia is very successful, it can be a long road. Treatment typically lasts two and a half to three and a half years, although the first three to six months are the most intense. During maintenance phases, kids can usually live a relatively normal life and go back to school. And adults may be able to continue working. Still, you'll likely need some tips for coping just as much as you need an explanation of the tests and treatment options:

• Get down to particulars. The term "leukemia" can be confusing, because it refers to a group of cancers that aren't all that similar except for the fact that they affect the bone marrow and blood. You can waste a lot of time gathering information that doesn't apply to you or your child.

  To avoid that, ask your doctor to write down as much information about your specific disease as possible. Then narrow your search for information accordingly. Write down questions you want to ask your doctor before each appointment, and look for information in your local library and on the Internet.

  Good sources include the National Cancer Institute, the American Cancer Society, and the Leukemia and Lymphoma Society. Also, consider bringing a tape recorder to appointments. Your doctor may present you with a lot of details in a single visit, and it can be helpful to capture those details and play them back later.

• Lean on your whole health care team. At major medical centers and pediatric cancer centers, your health care team may include psychologists, psychiatrists, recreation therapists, child-life workers, teachers, dietitians, chaplains and social workers. These professionals can help with a whole host of issues, including explaining procedures to children, finding financial assistance and arranging housing for during treatment. Don't hesitate to rely on their expertise.
• Explore programs for children with cancer. Major medical centers and nonprofit groups offer numerous activities and services specifically for children with cancer and their families. Examples include summer camps, support groups for siblings and wish-granting programs. Ask your health care team about programs in your area
• Help family and friends understand your situation. Set up a free, personalized Web page at the not-for-profit Web site CaringBridge. This allows you to tell the whole family about appointments, treatments, setbacks and reasons to celebrate — without the stress of calling everyone every time there's something new to report. A Web page helps your family and friends understand what you're going through, and it allows them to send notes of encouragement, even when they live far away.