Understanding ALL
Causes and Risk Factors
Signs and Symptoms
Diagnosis 
Treatment
Ph-Positive ALL-Induction /Post-Induction  Sides Effects of Treatment
Relapsed or Refractory ALL
Clinical Trials
Talk to the Doctor
Get More Information
Contact Us 
Helpful Links

Understanding ALL

Acute lymphocytic leukemia (ALL) is a type of blood cancer. Other names for ALL are acute lymphoblastic leukemia and acute lymphoid leukemia.

About 5,430 people in the United States are expected to be diagnosed with ALL in 2008. It is the most common type of leukemia in children under age 15. The risk of getting ALL increases in people ages 45 and older. However, people can get ALL at any age.

Most children with ALL are cured of their disease after treatment.

Causes and Risk Factors

ALL starts with a change to a single cell in the bone marrow. Scientists are studying the exact genetic changes that cause a normal cell to become an ALL cell. 

Few factors have been associated with an increased risk of developing ALL. Exposure to high doses of radiation therapy used to treat other types of cancer is one known risk factor. Other possible risk factors are continually under study. ALL is not contagious (catching).

ALL occurs at different rates in different geographic locations. There are higher rates in more developed countries and in higher socioeconomic groups. Scientists continue to explore possible relationships with life-style or environmental factors but no firm conclusions have yet been reached. This suggests that many factors may be involved. At the present time there is no known way to prevent most cases of the disease.

Information About Phosphocol P32.  Phosphocol P32 is a prescription drug approved to treat adults with fluid in the abdominal or chest cavity caused by  cancer or infection. Safety and effectiveness in children has not been established. The United States Food & Drug Administration (FDA) updated the safety information of this drug in August 2008 following reports linking Phosphocol P32 to leukemia, when used in an unapproved way to treat children with bleeding between the joints caused by hemophilia. The labeling of  Phosphocol P 32 was modified by the manufacturer, Covidien Ltd. in August 2008 to reflect this risk. Information about the leukemia cases [two children (ages 9 and 14) with hemophilia developed acute lymphocytic leukemia approximately 10 months after intra-articular injections of Phosphocol P 32 (0.6 and 1.5 mCi total dose] was added  to the "Warnings" section of the Phosphocol P 32 label. Also, "leukemia in children" is now noted as a risk in the label's "Adverse Events" section. Safety information is posted on the FDA Medwatch Web site (August 2008). The manufacturer strongly encourages medical professionals and their patients to follow the guidelines outlined in the prescribing information included with Phosphocol P 32. To read Covidien's communication to physicians click here August 29, 2008 manufacturer's letter to physicians.

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Signs and Symptoms

Some signs or symptoms of ALL are similar to other more common and less severe illnesses. Specific blood tests and bone marrow tests are needed to make a diagnosis. A person with ALL may have:

The best advice for any person troubled by symptoms such as a lasting, low-grade fever, unexplained weight loss, tiredness or shortness of breath is to see a healthcare provider.

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Diagnosis

Blood and bone marrow tests are done to look for leukemia cells. A CBC (complete blood count) is used to help diagnose ALL. A bone marrow aspirate and a bone marrow biopsy are two of the tests that are done. An aspirate is done to take a close look at the cells in the marrow in order to look for abnormal cells such as leukemic blast cells. It can also be used for cytogenetic analysis, immunophenotyping and other tests. The biopsy gives information about how much disease is in the marrow. Immunophenotyping is used to find out if the patient's leukemia cells are B cells or T cells. Most people with ALL have the B-cell type. Most cases of the B-cell type are called precursor B-cell type.

The doctor uses information from these tests to decide the type of drug therapy a patient needs and how long treatment will last. Bone marrow tests are also done to see if treatment is destroying leukemic blast cells.

To decide the best treatment for the patient, the doctor may also consider:

• The patient's age
• The number of ALL cells in the blood
• If the ALL has spread to the covering of the brain or spinal cord
• If there are certain chromosomal changes.

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Treatment

Patients with ALL need to start chemotherapy right away. It is important to get medical care in a center where doctors are experienced in treating patients with ALL.

The goal of treatment for ALL is to cure the disease. Children with ALL are likely to be cured of their disease. The number of adult patients who have remissions has increased. The length of remissions in adults has improved.

There are two parts of treatment for ALL, called induction therapy and post-induction therapy. The aim of induction therapy is to:

• Kill as many ALL cells as possible
• Get blood counts back to normal
• And to get rid of all signs of the disease for an extended period of time.

This is called a remission.

Some drugs used to treat ALL are given by mouth. Other drugs are given by placing a catheter in a vein - usually in the patient's upper chest. During induction therapy most patients are treated with more than one drug and they may be given several drugs in combination. Each drug type works in a different way to kill the cells. Combining drug types can strengthen the effects of the drugs. Some of the drugs used to treat ALL are clofarabine, cytarabine, daunorubicin, methotrexate, mitoxantrone, cyclophosphamide, vincristine, pegaspargase, imatinib mesylate, prednisone and dexamethasone.

Patients with ALL often have leukemic cells in the lining of the spinal cord and brain. The procedure used to check the spinal fluid for leukemic cells is called a spinal tap. The cells cannot always be found in an exam of the spinal fluid.

To prevent leukemia in the central nervous system (CNS) leukemia, all patients who are in remission have the lining of the spinal cord and brain treated. In some cases, treatment is needed for ALL that has already affected the lining of the spinal cord and brain (CNS leukemia) and is causing problems such as headache, nausea and vomiting, and blurred vision. Parts of the body that aren't easily reached with chemotherapy given by mouth or IV - such as the lining of the spinal cord and brain - are treated by injection into the spinal fluid. Drugs such as methotrexate or cytarabine are injected into the spinal fluid either to prevent or treat CNS leukemia.

When the treatment is for CNS leukemia, a spinal tap is done. Then spinal fluid is removed and chemotherapy is injected into the spinal canal.

Radiation therapy may be given to the spine or brain. Spinal taps are done from time to time to check if leukemic cells are being killed and to give more doses of chemotherapy. Sometimes both chemotherapy and radiation therapy are used.

Many ALL patients build up uric acid in their blood from their disease. Uric acid is a chemical made in the body. The use of chemotherapy also increases the uric acid. A high level of uric acid can cause kidney stones. Patients with high uric acid levels may be given a drug called allopurinol (Aloprim®, Zyloprim®) by mouth or IV. Another drug used to treat high uric acid levels is called rasburicase (Elitek®).

Post-Induction Therapy
More treatment is needed even after a patient with ALL is in remission. This is called post-induction therapy. It is given in cycles for two to three years. Post-induction therapy is given because some ALL cells remain that are not found by common blood or marrow tests. For most people, the postremission therapy drugs used are not the same drugs used during induction therapy. The doctor considers many things to decide the kind of post-induction therapy a patient needs, such as:

• The patient's response to induction therapy.
• Whether the patient has certain chromosomal abnormalities.

High-risk types of ALL - such as T-cell ALL, infant ALL and adult ALL - are usually treated with higher doses of drugs during induction and post-induction therapy. One treatment plan is to use higher doses of drugs and give them for a longer time. Allogeneic stem cell transplant may be a good treatment for some high-risk ALL patients.

Ph-Positive ALL-Induction/Post-Induction

About one out of five adults with ALL and a small number of children with ALL have a type called Ph-positive (or Philadelphia-positive) ALL.

Ph-positive ALL may be treated with imatinib mesylate, also called Gleevec® or with other related drugs, such as dasatinib (Sprycel®) or nilotinib (Tasigna®). These drugs are given with chemotherapy. Gleevec® (or Sprycel® or Tasigna®) is given by mouth. Doctors are studying how well this treatment works in patients with Ph-positive ALL.

During post-induction therapy, Gleevec® (or another related drug) is given with other drugs. Usually people with Ph-positive ALL stay on Gleevec®  (or another related drug) after post-induction therapy is completed.

Allogeneic Stem Cell Transplant
Allogeneic stem cell transplant is a treatment used for some patients with ALL.
The main purpose of doing the transplant is to give strong doses of chemotherapy or radiation therapy to kill the ALL cells. This will also kill the healthy stem cells in the marrow. The transplanted donor stem cells help start a new supply of red cells, white cells and platelets.

Allogeneic stem cell transplant is a high-risk procedure. For this reason, it may not be a good treatment for some ALL patients. Allogeneic stem cell transplant may be a choice for adult ALL patients if:

They are not doing well with other treatments. The expected benefits of stem cell transplant exceed the risks. There is a donor.

Stem cell transplant is usually not considered for a child unless:

• Doctors have determined that the child's type of ALL is not likely to respond well to chemotherapy.
• Chemotherapy has not worked well.
• The child has relapsed ALL.

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Side Effects of Treatment

Not all patients have treatment side effects. Patients who experience side effects should speak to their treatment teams about how to manage their side effects.

Possible side effects of treatment for ALL include:

• The number of red cells may decrease (called anemia). Transfusions of red cells (blood cells that are donated and given to the patient) may be needed to increase red cells.
• Patients also may have a drop in the number of platelets. If a patient's platelet count is very low he or she may need a platelet transfusion to prevent bleeding.
• A big drop in white cells may lead to an infection. Such infections are usually treated with antibiotics, until the white cell count goes up and the infection clears up. For adults, growth factors are sometimes given to increase white cells. G-CSF (Neulasta® or Neupogen®) and GM-CSF (Leukine®) are drugs that increase the number of white cells. The doctor may talk about the absolute neutrophil count or ANC, which is the number of neutrophils, a type of white cell a person has to fight an infection. Fever or chills may be the only signs of infection. Patients with an infection may also have:
  • Coughing
  • Sore throat
  • Pain when urinating
  • Frequent loose bowel movements.

To lower the risk of infection:

• The patient, the patient's visitors and medical staff need to wash their hands well.
• The patient's central line must be kept clean. Patients on chemotherapy should take good care of their teeth and gums.

Chemotherapy affects the parts of the body where new cells form quickly. This includes the inside of mouth and bowel, and the skin and hair. Some other chemotherapy side effects are:

• Mouth sores
• Diarrhea
• Hair loss
• Rashes
• Nausea
• Vomiting.

Drugs and other therapies can be given to prevent or treat nausea or vomiting.

Follow-up Visits
Patients who have finished all of their therapy still need to go to their doctors regularly for exams and tests. The doctor may recommend longer periods of time between follow-up visits if a patient continues to be disease free.

Treatment for ALL can cause long-term or late effects. Children should be checked for treatment effects on growth or learning that may not take place right away. It is important to identify problems early. Talk to the doctor about when your child's learning skills should be assessed. Some children will need special help with schoolwork during and after treatment.

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Relapsed or Refractory ALL

Some patients have a remission after treatment but then ALL cells return later - this is called a relapse. Other patients with ALL may still have ALL cells in the marrow even after treatment (refractory leukemia). 

For patients who relapse, the same or different drugs may be given, or be used. A drug called clofarabine (Clolar®) is being used to treat some children (ages 1 to 21) with relapsed and refractory ALL.

In refractory leukemia, drugs that were not used to treat the patient's ALL in the first round of treatment may be given. Allogeneic stem cell transplantation also may be used.

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Clinical Trials

Clinical trials are used to study new drugs, new treatments or new uses for approved drugs or treatments. These are some of the types of trials under way:

Leukemia-specific therapy, based on a patient's specific type of leukemia - such as the type of chromosome changes - is being studied.

The ALL cells of some patients are not as easily killed by drugs as those of other patients. This is called drug resistance. Scientists are trying to understand why some ALL cells are resistant to the effects of chemotherapy. This will help them develop better treatments.

Scientists are studying ways to boost the body's natural defenses, called immunotherapy. The goal is to kill or prevent the growth of ALL cells.

Blood cell growth factors can be used to help restore normal blood cells during treatment.

Scientists are studying the exact genetic changes that cause a normal cell to become an ALL cell. This research is leading to the development of new treatments. These treatments could block the effects of cancer-causing genes called oncogenes.

Gene profiling will be used more in the future to design more specific treatments for the different types of leukemia. New targeted treatments are being developed for ALL.

Many therapies, such as nilotinib, are being studied in clinical trials for Ph-positive ALL and other high-risk types of ALL. T-cell ALL, infant ALL and adult ALL are other high-risk types of ALL.

Doctors are studying a type of stem cell transplant, called a nonmyeloablative stem cell transplant (also called a reduced-intensity transplant).

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Talk to the Doctor

It may be helpful to write down questions to ask your doctor. You can also write down or record your doctor's answers and review them later. You may want to bring a family member or friend with you to the doctor. This person can listen, take notes and offer support. Some patients record information and listen to it at home.

• Questions to Ask Your Doctor
• Find Support

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Get More Information

Further details of treatment and supportive care and the beneficial and adverse effects of treatment may be obtained from the free LLS informational booklets Acute Lymphocytic Leukemia and Acute Lymphocytic Leukemia: A Guide for Patients and Caregivers.

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Contact Us

or call the Information Resource Center at (800) 955-4572.

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Helpful Links