Lymphoma

Lymphomas are blood cancers that develop in the immune system, more specifically in the lymphatic system- part of the body’s germ-fighting network and includes lymph nodes, spleen, thymus gland and bone marrow. It’s not clear what causes lymphoma, but it begins when a disease-fighting white blood cell called a lymphocyte develops a genetic mutation.

This mutation tells the lymphocyte cell to multiply rapidly, creating more and more mutated cells. The mutation also allows the mutated cells to go on living when normal cells would normally die. This results in a collection of too many diseased and infected lymphocytes in the lymph nodes, causing the lymph nodes to swell.

Common places for these lymph nodes to swell are in the neck, armpits and groin. Other signs and symptoms of lymphoma include persistent fatigue, fever, night sweats, shortness of breath and unexplained weight loss.

Many subtypes of lymphoma exist, but there are two main categories:

Hodgkin’s Lymphoma: mostly affects young adults and can be found almost anywhere in the body. This disease has subtypes, but recent advances in diagnosis and treatment have given patients the chance for a full recovery.

Non-Hodgkin’s Lymphoma (NHL): is the most common type of lymphoma, with 74,000 cases (4% of all cancers in the U.S.) diagnosed each year. There are many subtypes of NHL and treatment will vary depending on the diagnosis.

After you have been diagnosed with lymphoma, your physician will determine the stage of your cancer. Because there are so many different types of lymphoma, an accurate diagnosis is key to developing a treatment plan. The most common tests and procedures used in a diagnosis and staging include a physical exam, a lymph node biopsy, blood tests, a bone marrow biopsy and imaging tests such as CT, MRI and PET.

Stages of Lymphoma

• Stage 1: Cancer in this stage has one of these two characteristics: (1) Lymphoma cells are in one lymph node region, including either one lymph node or one group of adjacent lymph node; (2) Lymphoma cells are in one part of one organ outside of the lymphatic system. 
• Stage 2: Cancer in this stage has one of these two characteristics: (1) Lymphoma cells are found in two or more lymph node regions located on the same side of the diaphragm; (2) Lymphoma cells are found in a group of lymph nodes and one area of a nearby organ. Other lymph nodes on the same side of the diaphragm may also contain lymphoma cells.
• Stage 3: Cancer in this stage has one of these two characteristics: (1) Lymphoma cells are found in lymph nodes on both sides of the diaphragm; (2) Lymphoma cells are in lymph nodes above the diaphragm and in the spleen.
• Stage 4: Cancer in this stage has spread to at least one organ outside of the lymphatic system.

Treatment Options

Next, your cancer care team will help you develop a treatment plan that best fits your cancer and your overall goals for your health. The treatment your physician recommends may depend on the type of lymphoma you have and the stage your cancer is in. If you have any questions about your treatment options, always ask your doctor.

Treatment of lymphoma depends on the type and progression of the disease, but some treatment options include:

• Active surveillance: Some forms of lymphoma are very slow growing and it may be best to wait and treat the disease when it causes signs or symptoms that interfere with daily activities. Until then, patients may undergo periodic tests to monitor any progression.
• Chemotherapy: Drugs are usually administered through a vein, but can also be taken as a pill, depending on the specific drug being received. This is often an effective treatment for lymphoma because chemotherapy drugs enter through the blood stream and can reach the entire body.
• Immunotherapy: Used to either boost your immune system or to change the way your immune system works to target and fight the cancer cells. Monoclonal antibodies, which are man-made versions of the proteins made by your immune system, can be used to treat lymphoma. These drugs work by targeting a substance on the surface of lymphocytes, the cells where lymphoma starts. Immune checkpoint inhibitors may also be used to treat lymphoma. Your immune system keeps itself from attacking normal cells in the body with “checkpoint” proteins, which determine whether an immune response is started. Cancer cells can use these checkpoints and avoid being targeted by the immune system. Immunotherapy drugs target these checkpoints to start an immune response. Certain types of non-Hodgkin lymphoma may also be treated with drugs called bispecific antibodies, which attach to both cancer cells and immune system cells. This brings the two cells together, helping the immune system destroy the cancer cells. Chimeric antigen receptor (CAR) T-cell therapy may be used for some non-Hodgkin lymphomas. This treatment removes immune cells called T-cells from the patient’s blood and alters them in a lab. The altered cells have receptors that attach to the proteins on the surface of lymphoma cells to destroy them. 
• Stem Cell (or Bone Marrow) Transplant: May be used to treat lymphomas that have not responded to chemotherapy or radiation, or lymphoma that has come back after treatment. This procedure replaces diseased bone marrow with healthy bone marrow. Before a stem cell transplant, high doses of chemotherapy or radiation therapy will be given to destroy diseased bone marrow. Then an infusion of blood-forming stem cells is given to help rebuild healthy bone marrow. Stem cells may be from a donor or in some cases the patient’s own stem cells.
• Radiation therapy: Uses high-powered beams of energy, such as X-rays, to kill cancer cells. This therapy may be used to treat classic Hodgkin lymphoma at an early stage, often after chemotherapy. It may also be used for cancers at an advanced stage that have not responded to chemotherapy. Radiation is sometimes used by itself to treat nodular lymphocyte–predominant Hodgkin lymphoma found at an early stage. Radiation therapy can be used as the main treatment for some types of non-Hodgkin lymphoma. These cancers found at an early stage often respond well to radiation. If the cancer is found at a more advanced stage, radiation may be used along with chemotherapy. If you are receiving a stem cell transplant, radiation may be used to treat the whole body along with chemotherapy to eliminate lymphoma cells before the transplant. Radiation therapy is also used to treat symptoms caused by non-Hodgkin lymphoma that has spread to other organs, like the brain or spinal cord.

Risk factors for lymphoma include being a male over 65 years old, having an impaired immune system and having infections such as Espstein-Barr or Helicobacter pylori.