Non-Hodgkin Lymphoma

Recent data from the Leukemia and Lymphoma Society report that there are currently over 628,000 people in the United States living with some form of lymphoma. This includes over 474,000 diagnoses of non-Hodgkin lymphoma, and over 153,000 diagnoses of Hodgkin lymphoma (HL).

Hodgkin lymphoma is distinguished from other diseases classified as lymphoma primarily because of the presence of Reed-Sternberg cells, which are large, cancerous cells found in Hodgkin lymphoma tissues. Non-Hodgkin lymphomas (NHL) represent a varied group of diseases distinguished by the features of the cancerous cells with each disease type.

Non-Hodgkin lymphomas can stem from B cells or T cells. They can affect multiple parts of the the lymphoid tissue, including the lymph nodes, spleen, and the bone marrow and are further classified into fast-growing or slow-growing. There have been over sixty different subtypes of NHL identified, and 6 subtypes of HL, and those numbers are increasing.

Generally, the symptoms of NHL and HL are non-specific, most commonly including shortness of breath (from lymphoma affecting the thymus or nodes around the chest), abdominal pain and swelling which can cause loss of appetite, nausea and vomiting, fatigue, fever, lymphadenopathy (swelling of the lymph nodes), drenching night sweats, intense itching of the body for unknown reason, enlargement of the spleen, and weight loss. Lymphoma in the brain can cause headaches, problems with concentration, seizures and personality changes.

The causes of most lymphomas are unknown. Most are likely caused by genetic mutations. Environmental exposure to some pesticides, chemicals and herbicides have been implicated as well. People who have autoimmune diseases, organ transplants and weakened immune systems (such as AIDS) are also at greater risk. Some viruses have been identified as causative as well, although these are regionally specific.

The cornerstone of diagnosis is pathological examination of lymphatic tissue under a microscope. A biopsy of an enlarged lymph node or bone marrow will be taken and examined to figure out the specific type of cancer based on several tissue biological markers as well as cell structure. Additionally, blood tests, CT scans of the body, and PET scans may be done to see if the cancer has metastasized.

Treatment is dependent on many factors, most importantly the type of lymphoma diagnosed, followed by the stage of the cancer, the patient's age and health status, and how symptoms have affected the body. Typically treatment involves a wide variety of chemotherapeutic agents given in specific doses and combinations for the specific lymphoma. Radiation is frequently used as well, and is usually the primary treatment for non-Hodgkin lymphoma. Additionally, many new treatments called monoclonal antibodies have been successfully used (rituximab (Rituxan) for B-cell lymphomas, ofatumumab (Arzerra) for NHL, Ocrelizumab is in clinical trials). Some patients may benefit from a bone marrow transplant.

Prognosis for the various types of lymphoma vary depending on type and stage of advancement. While each type of lymphoma has its own prognosis, it is generally a very treatable illness, with over 80% of patients diagnosed early being alive after five years. Low-grade NHL usually progresses slowly, and sometimes it takes years for the disease to progress or even need treatment. Many types of high grade disease can be completely cured with chemotherapy. Unfortunately, if chemotherapy doesn't work, the disease can spread quickly.

Non-Hodgkin lymphoma can cause the following symptoms:

• swollen, painless lymph nodes in the neck, armpits, or groin
• unexplained weight loss
• fever
• soaking night sweats
• coughing, trouble breathing, or chest pain
• weakness and tiredness that don't go away
• pain, swelling, or a feeling of fullness in the abdomen

Most often, these symptoms are not due to cancer. Infections or other health problems may also cause these symptoms. Anyone with symptoms that do not go away within 2 weeks should see a doctor so that problems can be diagnosed and treated.

Doctors seldom know why one person develops non-Hodgkin lymphoma and another does not. But research shows that certain risk factors increase the chance that a person will develop this disease.

In general, the risk factors for non-Hodgkin lymphoma are mentioned below.

• Weakened immune system: The risk of developing lymphoma may be increased by having a weakened immune system (such as from an inherited condition or certain drugs used after an organ transplant).
• Certain infections:Having certain types of infections increases the risk of developing lymphoma. However, lymphoma is not contagious. You cannot catch lymphoma from another person.
• Hepatitis C virus: Some studies have found an increased risk of lymphoma in people with hepatitis C virus. More research is needed to understand the role of hepatitis C virus.
  Age: Although non-Hodgkin lymphoma can occur in young people, the chance of developing this disease goes up with age. Most people with non-Hodgkin lymphoma are older than 60. 

The following are the main types of infection that can increase the risk of lymphoma:

• Human immunodeficiency virus (HIV). HIV is the virus that causes AIDS. People who have HIV infection are at much greater risk of some types of non-Hodgkin lymphoma.
• Epstein-Barr virus (EBV). Infection with EBV has been linked to an increased risk of lymphoma. In Africa, EBV infection is linked to Burkitt lymphoma.
• Helicobacter pylori. H. pylori are bacteria that can cause stomach ulcers. They also increase a person's risk of lymphoma in the stomach lining.
• Human T cell leukemia/lymphoma virus type 1 (HTLV-1). Infection with HTLV-1 increases a person's risk of lymphoma and leukemia.
• Researchers are studying obesity and other possible risk factors for non-Hodgkin lymphoma. People who work with herbicides or certain other chemicals may be at increased risk of this disease. Researchers are also looking at a possible link between using hair dyes before 1980 and non-Hodgkin lymphoma.

Having one or more risk factors does not mean that a person will develop non-Hodgkin lymphoma. Most people who have risk factors never develop cancer.

If you have swollen lymph nodes or another symptom that suggests non-Hodgkin lymphoma, your doctor will try to find out what's causing the problem. Your doctor may ask about your personal and family medical history. You may have some of the following exams and tests.

• Physical exam: Your doctor checks for swollen lymph nodes in your neck, underarms, and groin. Your doctor also checks for a swollen spleen or liver.
• Blood tests: The lab does a complete blood count to check the number of white blood cells. The lab also checks for other cells and substances, such as lactate dehydrogenase (LDH). Lymphoma may cause a high level of LDH.
• Chest x-rays: You may have x-rays to check for swollen lymph nodes or other signs of disease in your chest.
• Biopsy: A biopsy is the only sure way to diagnose lymphoma. Your doctor may remove an entire lymph node (excisional biopsy) or only part of a lymph node (incisional biopsy). A thin needle (fine needle aspiration) usually cannot remove a large enough sample for the pathologist to diagnose lymphoma. Removing an entire lymph node is best. The pathologist uses a microscope to check the tissue for lymphoma cells.

It is important for you to take care of yourself by eating well and staying as active as you can.

You need the right amount of calories to maintain a good weight. You also need enough protein to keep up your strength. Eating well may help you feel better and have more energy.

Sometimes, especially during or soon after treatment, you may not feel like eating. You may be uncomfortable or tired. You may find that foods do not taste as good as they used to. In addition, the side effects of treatment (such as poor appetite, nausea, vomiting, or mouth sores) can make it hard to eat well. Your doctor, a registered dietitian, or another health care provider can suggest ways to deal with these problems.

Many people find they feel better when they stay active. Walking, yoga, swimming, and other activities can keep you strong and increase your energy. Exercise may reduce nausea and pain and make treatment easier to handle. It also can help relieve stress. Whatever physical activity you choose, be sure to talk to your doctor before you start. Also, if your activity causes you pain or other problems, be sure to let your doctor or nurse know about it.

Non-Hodgkin lymphoma and its treatment can lead to other health problems. You may receive supportive care to prevent or control these problems and to improve your comfort and quality of life during treatment.

You may receive antibiotics and other drugs to help protect you from infections. Your health care team may advise you to stay away from crowds and from people with colds and other contagious diseases. If an infection develops, it can be serious, and you will need treatment right away.

Non-Hodgkin lymphoma and its treatment also can lead to anemia, which may make you feel very tired. Drugs or blood transfusions can help with this problem.

If you have indolent non-Hodgkin lymphoma without symptoms, you may not need treatment for the cancer right away. The doctor watches your health closely so that treatment can start when you begin to have symptoms. Not getting cancer treatment right away is called watchful waiting.

If you have indolent lymphoma with symptoms, you will probably receive chemotherapy and biological therapy. Radiation therapy may be used for people with Stage I or Stage II lymphoma.

If you have aggressive lymphoma, the treatment is usually chemotherapy and biological therapy. Radiation therapy also may be used.

Watchful Waiting

People who choose watchful waiting put off having cancer treatment until they have symptoms. Doctors sometimes suggest watchful waiting for people with indolent lymphoma. People with indolent lymphoma may not have problems that require cancer treatment for a long time. Sometimes the tumor may even shrink for a while without therapy. By putting off treatment, they can avoid the side effects of chemotherapy or radiation therapy.

If you and your doctor agree that watchful waiting is a good idea, the doctor will check you regularly (every 3 months). You will receive treatment if symptoms occur or get worse.

Chemotherapy

Chemotherapy for lymphoma uses drugs to kill lymphoma cells. It is called systemic therapy because the drugs travel through the bloodstream. The drugs can reach lymphoma cells in almost all parts of the body.

You may receive chemotherapy by mouth, through a vein, or in the space around the spinal cord. Treatment is usually in an outpatient part of the hospital, at the doctor's office, or at home. Some people need to stay in the hospital during treatment.

Chemotherapy is given in cycles. You have a treatment period followed by a rest period. The length of the rest period and the number of treatment cycles depend on the stage of your disease and on the anticancer drugs used.

Biological Therapy

People with certain types of non-Hodgkin lymphoma may have biological therapy. This type of treatment helps the immune system fight cancer.

Monoclonal antibodies are the type of biological therapy used for lymphoma. They are proteins made in the lab that can bind to cancer cells. They help the immune system kill lymphoma cells. People receive this treatment through a vein at the doctor's office, clinic, or hospital.

Radiation Therapy

Radiation therapy (also called radiotherapy) uses high-energy rays to kill lymphoma cells. It can shrink tumors and help control pain. Two types of radiation therapy are used for people with lymphoma:

• external radiation. A large machine aims the rays at the part of the body where lymphoma cells have collected. This is local therapy because it affects cells in the treated area only. Most people go to a hospital or clinic for treatment 5 days a week for several weeks.
• systemic radiation. Some people with lymphoma receive an injection of radioactive material that travels throughout the body. The radioactive material is bound to monoclonal antibodies that seek out lymphoma cells. The radiation destroys the lymphoma cells.

Stem Cell Transplantation

If lymphoma returns after treatment, you may receive stem cell transplantation. A transplant of your own blood-forming stem cells allows you to receive high doses of chemotherapy, radiation therapy, or both. The high doses destroy both lymphoma cells and healthy blood cells in the bone marrow.

Stem cell transplants take place in the hospital. After you receive high-dose treatment, healthy blood-forming stem cells are given to you through a flexible tube placed in a large vein in your neck or chest area. New blood cells develop from the transplanted stem cells.

The stem cells may come from your own body or from a donor.

Taking Part in Cancer Research

Doctors all over the country are studying new ways to treat lymphoma. Clinical trials (research studies in which people volunteer to take part) find out whether promising approaches to treatment are safe and effective. Research already has led to advances.

Researchers are studying many types of treatments for lymphoma.

• Chemotherapy: Doctors are testing new drugs that kill cancer cells. They are working with many drugs and drug combinations. They also are looking at ways of combining drugs with other treatments, such as biological therapy.
• Radiation therapy: Doctors are testing radiation treatment alone and with chemotherapy.
• Biological therapy: New types of biological therapy are under study. For example, researchers are making cancer vaccines that may help the immune system kill lymphoma cells. Also, doctors are studying a type of biological therapy that delivers radiation directly to cancer cells.
• Stem cell transplantation: Doctors are studying stem cell transplantation in people with newly diagnosed lymphoma and those who have already been treated.

People who join clinical trials may be among the first to benefit if a new approach is effective. And even if participants do not benefit directly, they still help doctors learn more about lymphoma and how to control it. Although clinical trials may pose some risks, researchers do all they can to protect their patients.