Acute Lymphoblastic Leukemia (ALL)

What is acute lymphoblastic leukemia (ALL)?

Acute lymphoblastic leukemia (acute lymphocytic leukemia, ALL) is a rare blood cancer that affects a type of white blood cell called lymphocytes. ALL may affect anyone at any age, but children younger than 15 and adults older than 50 are more likely to develop the condition.

While ALL is a serious condition, thanks to newer treatments, including long-term chemotherapy, children with the condition can be cured, and other people are living longer with ALL.

Is ALL a common condition?

No, it’s not. Acute lymphocytic leukemia accounts for less than half of 1% of all cancers in the U.S. However, ALL is the most common cancer that affects children, teenagers and young adults. Most ALL cases affect children ages 2 to 5.

Where does ALL start?

It starts in your bone marrow, the spongy center of most bones that produces blood cells and platelets. Your bone marrow usually makes a carefully calibrated number of red blood cells, white blood cells and platelets that remain in your bone marrow until they mature and move into your bloodstream.

ALL affects your lymphocytes, a type of white blood cell that helps your body fight viruses and bacteria. Normally, your bone marrow produces immature white blood cells (lymphoblasts) that mature into healthy lymphocytes.

In ALL, leukemic lymphoblasts never mature. Instead, they multiply, crowding out other blood cells and platelets before moving from your bone marrow to your bloodstream and then to other areas of your body. As a result, your platelet levels are low and you’re likely to bruise more easily, bleed more than usual or develop anemia.

What are the types of acute lymphoblastic leukemia?

The two main types are B-cell ALL and T-cell ALL, named for the blood cells affected by ALL:

• B-cell ALL affects your B-cells, which make antibodies and help fight infection. B-cell ALL accounts for 75% to 80% of ALL cases.
• T-cell ALL affects your T-cells, which destroy germs and support other immune system cells.

A third type, natural killer ALL, is very rare.

What are symptoms of ALL?

Most ALL symptoms come on suddenly and affect children and adults similarly. Common initial symptoms include:

• Anemia.
• Bleeding, such as frequent nosebleeds or heavy menstrual periods.
• Bruising.
• Cough.
• Dizziness.
• Fatigue.
• Fever.
• Frequent viral infections or bacterial infections.
• Joint pain.
• Loss of appetite.
• Night sweats.
• Red, pinhead-sized spots on your skin (petechiae).
• Shortness of breath.
• Skin color that’s paler or lighter than usual.
• Swollen lymph nodes.
• Unexplained weight loss.
• Weakness.

Many acute lymphoblastic leukemia symptoms mimic other less serious conditions. Having one or more of the symptoms listed above isn’t a sign that you have ALL. In general, you should talk to a healthcare provider about changes in your body that last more than two weeks.

What causes acute lymphoblastic leukemia?

Researchers continue to find genetic mutations (changes) that cause ALL. Young children with ALL may have had genetic changes that happened before they were born. Some people have ALL because they inherited conditions that increase their chance of developing the disease. ALL in adults is linked to some carcinogens, including tobacco.

What are ALL risk factors?

Risk factors include:

• Sex. Girls age 1 and younger have a higher risk than boys of the same age. After age 1, the risk is higher for males.
• Race. People who are white are somewhat more likely to develop ALL than people who are Black.
• Exposure to radiation. Children exposed to radiation in utero have an increased risk of ALL. Likewise, children and adults who had radiation therapy for other cancers have increased ALL risk.
• Viruses. Some viral infections, including Epstein-Barr virus or human T-cell leukemia virus, increase your risk of developing ALL.

What inherited genetic conditions increase the risk of ALL?

People with the following inherited conditions have a higher risk of ALL than people who don’t have the conditions:

• Ataxia telangiectasia.
• Bloom syndrome.
• Down syndrome.
• Fanconi anemia.
• Klinefelter syndrome.
• Li-Fraumeni syndrome.
• Neurofibromatosis type-1 (NF1).
• Wiskott-Aldrich syndrome.

What are the complications of acute lymphoblastic leukemia?

ALL that spreads to your brain and spine may cause the following complications:

• Balance problems.
• Blurred vision.
• Facial muscle weakness or numbness.
• Headaches.
• Nausea and vomiting.
• Seizures.
• Enlarged liver (hepatomegaly).
• Enlarged spleen (splenomegaly).
• Superior vena cava (SVC) syndrome.

How is acute lymphoblastic leukemia diagnosed?

Your healthcare provider evaluates your symptoms, reviews your medical history and does a physical exam. If they suspect ALL, they may do the following tests, including tests to look for genetic changes:

• Complete blood count (CBC).
• Bone marrow biopsy.
• Lymph node biopsy.
• Lumbar puncture (spinal tap).
• Magnetic resonance imaging (MRI) scan.
• Computed tomography (CT) scan.
• Positron emission tomography (PET) scan.
• Flow cytometry to determine ALL sub-type.
• Cytogenetic tests to examine cells’ chromosomes.
• Molecular assays to check for certain genes, proteins or other molecules that may be signs of ALL.

How is ALL treated?

Healthcare providers may treat ALL with long-term chemotherapy, targeted therapy, immunotherapy or stem cells (bone marrow) transplantation. Adults and children with ALL may receive different types of cancer drugs and treatments.

Chemotherapy

Providers use chemotherapy as initial or front-line treatment for ALL. People with ALL receive chemotherapy in four phases. The treatment goal is to put ALL into complete remission. (Complete remission means treatment eliminates your symptoms, and tests show no sign of cancer.)

Chemotherapy for ALL takes place over several months and sometimes years, and typically involves high doses of cancer-killing drugs. People receiving chemotherapy for ALL should consider palliative care to help manage treatment side effects. ALL chemotherapy includes:

• Remission induction therapy, which destroys as many leukemia cells as possible, so ALL goes into complete remission. Typically, people remain in the hospital during remission induction therapy. This treatment takes place over four to six weeks. Studies show more than 95% of children and 75% to 80% of adults with ALL will have complete remission after remission induction therapy.
• Central nervous system-directed therapy to kill any leukemia cells in your central nervous system and keep ALL from spreading to your spinal fluid. (System-directed means you have chemotherapy that affects your entire body or system.)
• Consolidation therapy begins once ALL is in remission. This treatment works to destroy as many remaining cancerous cells as possible. Consolidation therapy involves being in the hospital for several months while you receive high-dose chemotherapy administered weekly.
• Continuation or maintenance therapy is a long-term treatment that may last two to three years. You don’t have to be in the hospital to receive maintenance therapy.

Targeted therapy

Targeted therapy focuses on specific genetic changes. About 25% of adults and some children with ALL have chromosomal mutations. Healthcare providers currently use tyrosine kinase (TKI) therapy to treat ALL in children and adults with a specific mutation called Philadelphia chromosome or t(9;22). TKI therapy blocks an enzyme zyme essential for ALL growth. TKI therapy kills ALL cells so your body gets back to normal blood cell production.

Immunotherapy

Immunotherapy helps your body’s own immune system attack cancer cells. Immunotherapy for ALL may include CAR-T cell therapy or monoclonal antibody therapy.

Radiation therapy

Healthcare providers may recommend radiation therapy to treat recurrent ALL or ALL that doesn’t respond to chemotherapy. Recently, providers have used radiation therapy to treat ALL that’s spread (metastasized) to people’s brains or spinal fluid.

Allogeneic stem cell (bone marrow) transplantation

When other treatments haven’t eliminated ALL, a healthcare provider may recommend allogeneic stem cell (bone marrow) transplantation to treat adults with acute lymphoblastic leukemia.

Can ALL be prevented?

No, it can’t. Children with ALL develop the condition because of genetic changes that happened before they were born. But adults with ALL may be able to lower their risk by avoiding carcinogens, including tobacco and toxic chemicals.

What is the prognosis for ALL?

Your prognosis is the outcome you may expect after treatment. ALL often goes into complete remission after chemotherapy that kills cancerous cells. In general, children and young adults have a better prognosis than do people age 20 and older.

What is the survival rate?

Acute lymphoblastic leukemia survival rates vary based on people’s ages. For example, studies show:

• More than 90% of children between birth and age 14 were alive five years after diagnosis.
• More than 70% of children age 15 to 19 were alive five years after diagnosis.
• More than 30% of people age 20 and older were alive five years after diagnosis.

When you think about survival rates, it’s important to remember that they’re estimates based on the experiences of people who have the same condition. Survival rates use data from the recent past, not the present. More than that, a survival rate estimate may not reflect your situation or your child’s situation. If you have questions about ALL survival rates, your healthcare provider is your best resource for information.

Is ALL curable?

In some cases, yes, ALL can be cured. Children with ALL who remain in complete remission after five years are considered cured. That’s because ALL rarely recurs (comes back) after five years. Older children and adults with ALL are less likely to be cured because treatment doesn’t always put ALL into long-term remission.

What’s it like to live with acute lymphoblastic leukemia?

Depending on your situation, living with lymphoblastic leukemia may be like living with a chronic disease:

• ALL is a rare condition. Not many people have it or know what it means to live with ALL. If you or your child have ALL, participating in support groups may help. (Along those lines, childhood cancer is rare. If your child has ALL, you and your child may benefit by working with child life specialists.)
• Many people with ALL need long-term chemotherapy to keep the condition in remission. Consider participating in cancer survivorship programs. (Anyone with cancer is a cancer survivor.) Survivorship programs have resources that help with issues like fear of cancer recurrence and living in remission from cancer.
• Children and adolescents treated for ALL will need follow-up care for the rest of their lives so providers can monitor for complications or late effects of cancer treatment. Late effects are medical issues that may develop years after people finish treatment.
• Smoking tobacco is a known ALL risk factor. If you’re an adult with ALL and you smoke tobacco, try to quit. If your adolescent child has ALL, consider explaining the risks of using tobacco.
• Cancer is stressful. Gentle exercise may help ease stress. Even young children may benefit from regular exercise to help them manage their feelings about having cancer.

What questions should I ask my healthcare provider?

If you or your child have acute lymphocytic leukemia, you may want to ask your healthcare provider the following questions:

• What type of ALL do I have?
• What are possible treatments?
• What are the chances initial treatment will put ALL into remission?
• What are treatment options if ALL comes back after treatment?
• Are there clinical trials I should consider?