Leukemia (Part 1)

What Is Leukemia?

Leukemia is a type of cancer. Cancer is a group of many related diseases. All cancers begin in cells, which make up blood and other tissues. Normally, cells grow and divide to form new cells as the body needs them. When cells grow old, they die, and new cells take their place.

Sometimes this orderly process goes wrong. New cells form when the body does not need them, and old cells do not die when they should. Leukemia is cancer that begins in blood cells. Normal Blood Cells Blood cells form in the bone marrow. Bone marrow is the soft material in the center of most bones. Immature blood cells are called stem cells and blasts. Most blood cells mature in the bone marrow and then move into the blood vessels. Blood flowing through the blood vessels and heart is called the peripheral blood. Diagram shows the pluripotent stem cells, branching off into myeloid and lymphoid stem cells, becoming blast cells, and then becoming red blood cells, white blood cells, or platelets.

Picture of blood cells maturing from stem cells.

The bone marrow makes different types of blood cells. Each type has a special function:

White blood cells help fight infection.

Red blood cells carry oxygen to tissues throughout the body.

Platelets help form blood clots that control bleeding.

Leukemia Cells

In people with leukemia, the bone marrow produces abnormal white blood cells. The abnormal cells are leukemia cells. At first, leukemia cells function almost normally. In time, they may crowd out normal white blood cells, red blood cells, and platelets. This makes it hard for blood to do its work.

Types of Leukemia

The types of leukemia are grouped by how quickly the disease develops and gets worse. Leukemia is either chronic (gets worse slowly) or acute (gets worse quickly):

• Chronic leukemia—Early in the disease, the abnormal blood cells can still do their work, and people with chronic leukemia may not have any symptoms. Slowly, chronic leukemia gets worse. It causes symptoms as the number of leukemia cells in the blood rises.
• Acute leukemia—The blood cells are very abnormal. They cannot carry out their normal work. The number of abnormal cells increases rapidly. Acute leukemia worsens quickly.

There are four common types of leukemia:

• Chronic lymphocytic leukemia (chronic lymphoblastic leukemia, CLL) accounts for about 7,000 new cases of leukemia each year. Most often, people diagnosed with the disease are over age 55. It almost never affects children.
• Chronic myeloid leukemia (chronic myelogenous leukemia, CML) accounts for about 4,400 new cases of leukemia each year. It affects mainly adults.
• Acute lymphocytic leukemia (acute lymphoblastic leukemia, ALL) accounts for about 3,800 new cases of leukemia each year. It is the most common type of leukemia in young children. It also affects adults.
• Acute myeloid leukemia (acute myelogenous leukemia, AML) accounts for about 10,600 new cases of leukemia each year. It occurs in both adults and children.

Hairy cell leukemia is a rare type of chronic leukemia. This booklet does not deal with hairy cell leukemia or other rare types of leukemia. Together, these rare leukemias account for about 5,200 new cases of leukemia each year. The Cancer Information Service (1-800-4-CANCER) can provide information about these types of leukemia.

Leukemia: Who’s at Risk?

No one knows the exact causes of leukemia. Doctors can seldom explain why one person gets this disease and another does not. However, research has shown that people with certain risk factors are more likely than others to develop leukemia. A risk factor is anything that increases a person’s chance of developing a disease.

Studies have found the following risk factors for leukemia:

• Very high levels of radiation—People exposed to very high levels of radiation are much more likely than others to develop leukemia. Very high levels of radiation have been caused by atomic bomb explosions (such as those in Japan during World War II) and nuclear power plant accidents (such as the Chernobyl [also called Chornobyl] accident in 1986).

  Medical treatment that uses radiation can be another source of high-level exposure. Radiation used for diagnosis, however, exposes people to much lower levels of radiation and is not linked to leukemia.

• Working with certain chemicals—Exposure to high levels of benzene in the workplace can cause leukemia. Benzene is used widely in the chemical industry. Formaldehyde is also used by the chemical industry. Workers exposed to formaldehyde also may be at greater risk of leukemia.
• Chemotherapy—Cancer patients treated with certain cancer-fighting drugs sometimes later develop leukemia. For example, drugs known as alkylating agents are associated with the development of leukemia many years later.
• Down syndrome and certain other genetic diseases—Some diseases caused by abnormal chromosomes may increase the risk of leukemia.
• Human T-cell leukemia virus-I (HTLV-I)—This virus causes a rare type of chronic lymphocytic leukemia known as human T-cell leukemia. However, leukemia does not appear to be contagious.
• Myelodysplastic syndrome—People with this blood disease are at increased risk of developing acute myeloid leukemia.

In the past, some studies suggested exposure to electromagnetic fields as another possible risk factor for leukemia. Electromagnetic fields are a type of low-energy radiation that comes from power lines and electric appliances. However, results from recent studies show that the evidence is weak for electromagnetic fields as a risk factor.

Most people who have known risk factors do not get leukemia. On the other hand, many who do get the disease have none of these risk factors. People who think they may be at risk of leukemia should discuss this concern with their doctor. The doctor may suggest ways to reduce the risk and can plan an appropriate schedule for checkups.

Symptoms

Like all blood cells, leukemia cells travel through the body. Depending on the number of abnormal cells and where these cells collect, patients with leukemia may have a number of symptoms.

Common symptoms of leukemia may include:

• Fevers or night sweats
• Frequent infections
• Feeling weak or tired
• Headache
• Bleeding and bruising easily (bleeding gums, purplish patches in the skin, or tiny red spots under the skin)
• Pain in the bones or joints
• Swelling or discomfort in the abdomen (from an enlarged spleen)
• Swollen lymph nodes, especially in the neck or armpit
• Weight loss

Such symptoms are not sure signs of leukemia. An infection or another problem also could cause these symptoms. Anyone with these symptoms should see a doctor as soon as possible. Only a doctor can diagnose and treat the problem.

In the early stages of chronic leukemia, the leukemia cells function almost normally. Symptoms may not appear for a long time. Doctors often find chronic leukemia during a routine checkup—before there are any symptoms. When symptoms do appear, they generally are mild at first and get worse gradually.

In acute leukemia, symptoms appear and get worse quickly. People with this disease go to their doctor because they feel sick. Other symptoms of acute leukemia are vomiting, confusion, loss of muscle control, and seizures. Leukemia cells also can collect in the testicles and cause swelling. Also, some patients develop sores in the eyes or on the skin. Leukemia also can affect the digestive tract, kidneys, lungs, or other parts of the body.

Diagnosis

If a person has symptoms that suggest leukemia, the doctor may do a physical exam and ask about the patient's personal and family medical history. The doctor also may order laboratory tests, especially blood tests.

The exams and tests may include the following:

• Physical exam—The doctor checks for swelling of the lymph nodes, spleen, and liver.
• Blood tests—The lab checks the level of blood cells. Leukemia causes a very high level of white blood cells. It also causes low levels of platelets and hemoglobin, which is found inside red blood cells. The lab also may check the blood for signs that leukemia has affected the liver and kidneys.
• Biopsy—The doctor removes some bone marrow from the hipbone or another large bone. A pathologist examines the sample under a microscope. The removal of tissue to look for cancer cells is called a biopsy. A biopsy is the only sure way to know whether leukemia cells are in the bone marrow.

There are two ways the doctor can obtain bone marrow. Some patients will have both procedures:

• Bone marrow aspiration: The doctor uses a needle to remove samples of bone marrow.
• Bone marrow biopsy: The doctor uses a very thick needle to remove a small piece of bone and bone marrow.

Local anesthesia helps to make the patient more comfortable.

• Cytogenetics—The lab looks at the chromosomes of cells from samples of peripheral blood, bone marrow, or lymph nodes.
• Spinal tap—The doctor removes some of the cerebrospinal fluid (the fluid that fills the spaces in and around the brain and spinal cord). The doctor uses a long, thin needle to remove fluid from the spinal column. The procedure takes about 30 minutes and is performed with local anesthesia. The patient must lie flat for several hours afterward to keep from getting a headache. The lab checks the fluid for leukemia cells or other signs of problems.
• Chest x-ray—The x-ray can reveal signs of disease in the chest.

A person who needs a bone marrow aspiration or bone marrow biopsy may want to ask the doctor the following questions:

• Will you remove the sample of bone marrow from the hip or from another bone?
• How long will the procedure take? Will I be awake? Will it hurt?
• How soon will you have the results? Who will explain them to me?
• If I do have leukemia, who will talk to me about treatment? When?

Treatment Many people with leukemia want to take an active part in making decisions about their medical care. They want to learn all they can about their disease and their treatment choices. However, the shock and stress after a diagnosis of cancer can make it hard to think of everything to ask the doctor. Often it helps to make a list of questions before an appointment. To help remember what the doctor says, patients may take notes or ask whether they may use a tape recorder. Some also want to have a family member or friend with them when they talk to the doctor—to take part in the discussion, to take notes, or just to listen. The doctor may refer patients to doctors who specialize in treating leukemia, or patients may ask for a referral. Specialists who treat leukemia include hematologists, medical oncologists, and radiation oncologists. Pediatric oncologists and hematologists treat childhood leukemia.

Whenever possible, patients should be treated at a medical center that has doctors experienced in treating leukemia. If this is not possible, the patient’s doctor may discuss the treatment plan with a specialist at such a center.

Getting a Second Opinion

Sometimes it is helpful to have a second opinion about the diagnosis and the treatment plan. Some insurance companies require a second opinion; others may cover a second opinion if the patient or doctor requests it. There are a number of ways to find a doctor for a second opinion:

• The patient’s doctor may be able to suggest a doctor who specializes in adult or childhood leukemia. At cancer centers, several specialists often work together as a team.
• The Cancer Information Service, at 1-800-4-CANCER, can tell callers about nearby treatment centers.

A local or state medical society, a nearby hospital, or a medical school can usually provide the names of specialists.

• The American Board of Medical Specialties (ABMS) has a list of doctors who have met certain education and training requirements and have passed specialty examinations. The Official ABMS Directory of Board Certified Medical Specialists lists doctors' names along with their specialty and their educational background. The directory is available in most public libraries. Also, ABMS offers this information on the Internet at http://www.abms.org. (Click on "Who's Certified.")

Preparing for Treatment

The doctor can describe treatment choices and discuss the results expected with each treatment option. The doctor and patient can work together to develop a treatment plan that fits the patient’s needs.

Treatment depends on a number of factors, including the type of leukemia, the patient’s age, whether leukemia cells are present in the cerebrospinal fluid, and whether the leukemia has been treated before. It also may depend on certain features of the leukemia cells. The doctor also takes into consideration the patient’s symptoms and general health.

These are some questions a person may want to ask the doctor before treatment begins:

• What type of leukemia do I have?
• What are my treatment choices? Which do you recommend for me? Why?
• What are the benefits of each kind of treatment?
• What are the risks and possible side effects of each treatment?
• If I have pain, how will you help me?
• What is the treatment likely to cost?
• How will treatment affect my normal activities?
• Would a clinical trial (research study) be appropriate for me? Can you help me find one?

People do not need to ask all of their questions or understand all of the answers at one time. They will have other chances to ask the doctor to explain things that are not clear and to ask for more information.

Methods of Treatment

The doctor is the best person to describe the treatment choices and discuss the expected results. Depending on the type and extent of the disease, patients may have chemotherapy, biological therapy, radiation therapy, or bone marrow transplantation. If the patient’s spleen is enlarged, the doctor may suggest surgery to remove it. Some patients receive a combination of treatments.

People with acute leukemia need to be treated right away. The goal of treatment is to bring about a remission. Then, when signs and symptoms disappear, more therapy may be given to prevent a relapse. This type of therapy is called maintenance therapy. Many people with acute leukemia can be cured.

Chronic leukemia patients who do not have symptoms may not require immediate treatment. The doctor may suggest watchful waiting for some patients with chronic lymphocytic leukemia. The health care team will monitor the patient’s health so that treatment can begin if symptoms occur or worsen. When treatment for chronic leukemia is needed, it can often control the disease and its symptoms. However, chronic leukemia can seldom be cured. Patients may receive maintenance therapy to help keep the cancer in remission.

A patient may want to talk to the doctor about taking part in a clinical trial, a research study of new treatment methods. The section on “The Promise of Cancer Research” has more information about clinical trials.

In addition to anticancer therapy, people with leukemia may have treatment to control pain and other symptoms of the cancer, to relieve the side effects of therapy, or to ease emotional problems. This kind of treatment is called symptom management, supportive care, or palliative care.