Leukemia and Lymphoma
Important Information You Should Know

Leukemia

The term leukemia comes from the Greek words for "white" (leukos) and "blood" (haima). Leukemia is a cancer (an abnormal growth of cells) of the blood and bone marrow. Unlike other cancers, leukemia does not produce a mass (tumor), but results in the overproduction of abnormal white blood cells.

Leukemia begins in the immature or developing cells of the bone marrow, the soft, spongy tissue found in the central cavities of bones. The bone marrow produces all types of blood cells: red blood cells that carry oxygen and other materials to the tissues of the body; white blood cells that fight infection; and platelets that help the blood clot. Hundreds of billions of new blood cells are produced in the bone marrow each day, providing the body with a constant supply of fresh, healthy cells.

In a patient with leukemia, many of the white blood cells produced in the bone marrow do not mature normally. These abnormal cells, called leukemic cells, are unable to fight infection the way healthy white cells can. As they grow in number, the leukemic cells also interfere with the production of other blood cells.

How common is leukemia?

Leukemia often is considered a disease of children, yet it actually affects far more adults. In fact, the frequency of the disease increases with age. Leukemia is more common in men than in women, and more common in Caucasians than in African-Americans. Almost 30,000 cases are diagnosed in the United States each year.

What are the types of leukemia?

There are many types of leukemia, which are classified by the specific type of white blood cell involved. White blood cells include neutrophils and monocytes, which ingest (eat) bacteria and other germs; eosinophils and basophils, which are involved in allergic reactions; and lymphocytes, which play a key role in our body's immune system.

The main types of leukemia are myelogenous and lymphocytic, and each type has an acute (rapidly progressing) and a chronic (slowly progressing) form. Acute leukemia mainly affects cells that are immature or not fully developed, preventing them from maturing and functioning normally. Chronic leukemia develops more slowly, so that the body still has some healthy cells available to fight infection.

The four main forms of leukemia are:

• Acute lymphocytic leukemia (ALL)


• Chronic lymphocytic leukemia (CLL)


• Acute myelogenous leukemia (AML)


• Chronic myelogenous leukemia (CML)

In addition, there also are various subtypes of leukemia. Subtypes of lymphocytic leukemia include hairy cell, Waldenstrom’s macroglobulinemia, prolymphocytic, and lymphoma cell leukemia. Among the subtypes of myelogenous leukemia are myelogenous, promyelocytic, monocytic, erythroleukemia, and megakaryocytic leukemia.

What are the symptoms of leukemia?

In many cases, people in the early stages of leukemia have no obvious symptoms. When symptoms do appear, they may include:

• Anemia. Anemia is caused by having a lower than normal number of red blood cells, which slows down the delivery of oxygen to the body's organs and muscles. A person with anemia may have a pale complexion and may tire easily and have little energy.


• Easy bruising or bleeding. People with leukemia may bleed from their gums or noses, or may find blood in their stool or urine. Bruises may develop from very minor bumps. Small spots of discoloration—called petechiae—may form under the skin.


• Susceptibility to infections. Because leukemia affects the body's infection-fighting cells, a person with this cancer may develop infections, such as a sore throat or bronchial pneumonia. A headache, low-grade fever, mouth sores or skin rash may accompany the infection.


• Swollen lymph nodes. Lymph nodes are small, bean-sized structures that contain clusters of lymphocytes. In a person with leukemia, abnormal lymphocytes may collect in lymph nodes in the throat, armpits or groin, causing the lymph nodes to become enlarged.

General loss of well-being. Other symptoms of leukemia include loss of appetite and weight, discomfort under the left lower ribs (caused by a swollen spleen, also from a collection of abnormal lymphocytes) and a feeling of weakness or fatigue all the time. In some cases, a person with leukemia may get a fever that lasts for more than 1 to 2 weeks and may have night sweats.

What causes leukemia?

Leukemia results when the DNA of a single cell in the bone marrow becomes damaged. This is called a mutation and changes the cell's ability to develop and function normally. Further, all cells that arise from that initial cell also have the mutated DNA. What causes the damage to the DNA in the first place, however, is still not known. (DNA is the material in a cell that holds the instruction codes for the cell's growth and function. Segments of DNA make up genes, which are arranged on larger structures called chromosomes.) Scientists have been able to locate changes in certain chromosomes of patients diagnosed with different types of leukemia.

Although the exact cause of the DNA mutation that leads to leukemia is unknown, scientists have discovered certain factors that may put a person at higher risk for developing a form of the disease. For example, very high doses of radiation, exposure to the chemical benzene and exposure to certain chemotherapy drugs may increase the risk of developing AML, ALL or CML.

People with certain genetic disorders, such as Down syndrome, may be at higher risk for AML.

Further, a specific genetic abnormality—called the Philadelphia chromosome (Ph) after the city in which it was first identified—has been found in the marrow and blood cells of people with CML.

How is leukemia diagnosed?

Because chronic leukemia shows no obvious symptoms in the early stages, the disease may be diagnosed during a regular physical examination or as a result of routine blood tests. If a patient has enlarged lymph nodes, swollen gums, an enlarged liver or spleen, significant bruising or a small pinpoint rash, the doctor may suspect leukemia. Many patients initially just feel overall severe fatigue or flu-like symptoms which linger and do not improve.

To diagnose leukemia, the doctor must examine cells from the blood and, in most cases, the bone marrow. An initial blood test (complete blood count [CBC]) showing an abnormal white cell count may indicate the need for a bone marrow biopsy to confirm the diagnosis and to identify the specific type of leukemia. During this procedure, the doctor removes a sample of bone marrow tissue (biopsy) from a pelvic bone and tests the sample for cancer cells. The cells also are examined for chromosomal abnormalities. This is called a cytogenetics analysis (cyto=cell).

How is leukemia treated?

Treatment varies with the type and stage of the disease. For acute leukemia, the immediate goal of treatment is remission, which means no more cancerous cells can be detected and the bone marrow appears normal. The patient undergoes aggressive chemotherapy (the use of drugs to kill cancer cells) in a hospital for about 4 weeks. Since acute leukemia patients have extremely low counts of healthy blood cells, they usually are given transfusions of red blood cells and platelets. They also receive drugs to fight infection, and to reduce nausea and vomiting that may occur as side effects of the chemotherapy.

• Acute lymphocytic leukemia. People with ALL are likely to attain satisfactory remission after several weeks of aggressive chemotherapy. To keep the disease under control, they will continue receiving low-dose chemotherapy and possibly radiation therapy (use of radiation to kill cancer cells) for a month or more to eliminate remaining traces of cancer. At home, they will receive an on-and-off maintenance treatment for months or years.


• Acute myelogenous leukemia. Several chemotherapy drugs are effectively used to treat AML. However, it has been found that the genetic makeup of the abnormal leukemia cells can vary. Treatment can be suited to a particular patient based on the analysis of genetic makeup. If the disease is resistant to treatment, the best chance of a lasting remission or cure depends on successful bone marrow transplantation.


• Chronic lymphocytic leukemia. CLL generally affects older people and progresses slowly. Conventional treatment tends to be conservative. As long as symptoms are absent, the disease requires no treatment. If swelling appears in lymph nodes and other organs, CLL usually can be controlled for years with chemotherapy. Many people with CLL lead basically normal lives and die of unrelated causes.


• Chronic myelogenous leukemia. Modern treatment has essentially cured CML although the medicine, in the form of pills, usually needs to be taken forever. Cure is possible even for those patients for whom the pills fail with bone marrow transplantation. What is the prognosis for people with leukemia?

The rate of leukemia has not changed much since the 1950s, but more people are surviving longer. ALL (childhood leukemia), for example, represents one of the most dramatic success stories in cancer treatment. Almost 90% of children diagnosed with the disease are cured. Adult patients treated for ALL have an 80% to 90% chance of attaining remission; about 40% of those who do so survive at least another five years, with a chance of a full cure. Patients treated for AML have a 60% to 70% chance of remission; about 30% of those survive at least three years, with a possibility of a full cure.

Adult patients treated for ALL have an 80 percent to 90 percent chance of attaining remission; about 40 percent of those who do so survive at least another five years, with a chance of a full cure. Patients treated for AML have a 60 percent to 70 percent chance of remission; about 20 percent of those survive at least three years, with a possibility of a full cure.

At this time, there is no known way to prevent leukemia.

Call your doctor if:

• You experience any of the symptoms listed in the description section and cannot readily explain their occurrence. In this instance, your blood cell count should be tested.


• You experience unexplained bleeding, high fever or a seizure. These signs may indicate that you may need emergency treatment for acute leukemia.


• You are in remission from leukemia and notice signs of recurrence, such as infection or easy bleeding. If so, you should have a follow-up examination.

Adult Non-Hodgkin’s Lymphoma

What is adult non-Hodgkin’s lymphoma?

Adult non-Hodgkin’s lymphoma (NHL) is a malignant cancer that begins in the lymph system. The lymph system is part of the body’s immune system. “Malignant” means a disease spreads from where it starts and invades other areas of the body. Adult non-Hodgkin’s lymphoma differs from lymphoma in children and has a different course of treatment. “Non-Hodgkin’s” means the absence of a certain type of cell, called Reed-Sternberg cells, in the cancerous tissue.

The lymph includes the following:

• Bone marrow – The spongy interior of large bones where white blood cells, red blood cells (which carry oxygen), and platelets (which help blood clot) are made.


• Lymph – Colorless, watery fluid that carries white blood cells (lymphocytes) through the vessels of the lymph system all through the body. Lymphocytes can be either B-cells or T-cells. Their job is to fight disease and the growth of tumors.


• Lymph vessels – Tubes that collect lymph from different parts of the body and send it back to the bloodstream.


• Lymph nodes – Small oval or round structures that filter lymph and store white blood cells. Lymph nodes are located throughout the body but are concentrated in the underarms, pelvis, neck, abdomen and groin.


• Thymus – An organ behind the breastbone in which lymphocytes grow and reproduce.


• Spleen – An organ on the left side of the body near the stomach. The spleen filters blood, stores blood cells, and removes old blood cells. It also makes lymphocytes.


• Tonsils – Lymph tissue in the back of the throat that makes lymphocytes.

Because the lymph system is present throughout the body, lymphoma can begin in almost any area and spread to other tissues and organs such as the liver.

What are the risk factors for non-Hodgkin’s lymphoma?

• Being older, male, or Caucasian


• Having any of the following conditions:


• An inherited immune system disorder


• An autoimmune disease, such as rheumatoid arthritis or psoriasis


• HIV/AIDS


• Human T-lymphotrophic virus Type I or Epstein-Barr virus


• Repeated bouts of Helicobacter pylori infection, which causes stomach ulcers


• Use of immunosuppressant drugs following an organ transplant


• Exposure to certain pesticides


• Exposure to radiation


• A diet containing high amounts of meat and fat


• Previous treatment for Hodgkin’s lymphoma


• What are the symptoms of adult non-Hodgkin’s lymphoma?


• Painless swelling in the lymph nodes of the neck, underarm, groin, or stomach


• Unexplained fever


• Heavy night sweats


• Feeling much more tired than usual for at least several weeks for no apparent reason


• Unexplained weight loss


• Skin rash or itchy skin


• Unexplained pain in the chest, abdomen, or bones

How common is adult non-Hodgkin’s lymphoma?

An estimated 70,800 people (38,270 men and 32,530 women) in the US were diagnosed with NHL in 2013, making it the sixth most common cancer in women and the seventh most common cancer in men.

How is adult non-Hodgkin’s lymphoma diagnosed?

• Physical examination and medical history, focusing especially on lumps or anything that seems unusual.


• Blood tests to measure levels of red blood cells, white blood cells, and platelets


• Blood and urine tests that measure the amounts of certain immunoglobulins (proteins produced by the immune system to help fight infections).


• Bone marrow aspiration or biopsy – Using a needle to take a sample of bone marrow, from the back of the pelvic bones. The sample is then viewed under a microscope for signs of cancer.


• Lymph node biopsy – Removal of all or part of a lymph node in order to examine it under a microscope for signs of cancer.


• If cancer is detected, more laboratory tests will be needed to pinpoint what specific form of the disease is present.

Can adult non-Hodgkin’s lymphoma be treated?

Yes, NHL is a very treatable disease. Before treatment begins, it is necessary to know how far the cancer has advanced. This is called the stage of the disease. The stages begin with I (least severe) and go through IV (most severe). Stages of adult non-Hodgkin lymphoma may include “E” (meaning extranodal, or that cancer has spread beyond the lymph nodes) and “S” (meaning that cancer has been found in the spleen).

Because NHL is a blood and lymph system disease, most patients are at an advanced stage at diagnosis.

NHL comes in many types, but is often divided into indolent (slow-moving, usually with few symptoms) or aggressive (growing quickly, often causing symptoms) These two forms of NHL behave quite differently and are treated differently.

How is adult non-Hodgkin’s lymphoma treated?

There are several types of treatments used to kill cancer cells or keep them from dividing:

• Radiation therapy – X-rays or other types of radiation.


• Chemotherapy – Drugs taken either by mouth or injected into a vein, or less commonly into the cerebrospinal fluid.


• Targeted therapy – Treatments such as monoclonal antibodies that bind to targets on the surface of lymphocytes or small molecules designed to block pathways that make lymphoma cells grow but are not critical to normal cells.


• Biologic therapy (also called biotherapy or immunotherapy) – Using substances or cells made by the patient’s own immune system or in a laboratory to help boost the body’s natural defenses.

Some patients with indolent lymphoma that is not causing problems do not need treatment. This is called watchful waiting. It is the close monitoring of the patient without giving any treatment until symptoms appear or change.

There are also a number of new treatments being developed in clinical trials. A clinical trial might be preferred if standard treatments do not work or are too toxic. Ask the doctor about clinical trials.

What can be expected after treatment for adult non-Hodgkin’s lymphoma?

Regular appointments with the patient’s doctor are necessary following any treatment for cancer. It is important to monitor how the patient feels and to do physical exams. Also, providers need to perform laboratory tests or imaging tests to look for signs of cancer or side effects from treatment.

For many people with NHL, treatment successfully removes the cancer. For others, the lymphoma may never completely disappear. Ongoing treatments will be needed as long as possible to keep the disease under control.

What is the outlook for people with adult non-Hodgkin’s lymphoma?

The outlook for people with this disease depends upon the stage of the cancer. Another factor is the type of lymphoma present. The age and general health of the patient also are taken into account. In general, the survival rate of patients with NHL at the five-year mark is more than 60%.

Other Facts:

• Indolent NHLs have a median survival rate of as long as 20 years, and Stage I and Stage II varieties can often be treated with radiation alone.


• When treated early, over half of patients with aggressive NHLs can be cured with combination chemotherapies.


• Most relapses of aggressive NHL occur within two years of treatment.


• Relapses of indolent lymphoma can occur later.

How can adult non-Hodgkin’s lymphoma be prevented?

Many of the risk factors for NHL are outside a person’s direct control. The following factors, however, can be controlled:

• Avoiding behaviors that increase the chance of getting AIDS and hepatitis C infections, both of which weaken the immune system.


• Avoiding unnecessary exposure to radiation.


• Avoiding exposure to chemicals such as benzene and certain herbicides and insecticides.


• Maintaining normal weight and eating a healthy diet.


• Rarely, some women develop lymphoma in the scar tissue around breast implants. The decision to get breast implants should be well thought-out.

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