Stages of Lymphoma
When lymphoma is found early, it tends to be confined to one or more lymph nodes. More advanced disease occurs when cancer cells have spread beyond the lymph system into organs like the liver, skin, brain, bone or other tissues.
Lymphoma stages include:
- Stage 1: One lymph node area, such as the side of the neck or underarm, is impacted.
- Stage 2: Two or more lymph node areas are impacted, but on the same side of the diaphragm — either all below or all above.
- Stage 3: More than one lymph node area on both sides of the diaphragm are impacted.
- Stage 4: The disease has spread beyond the lymphatic system, most commonly to the bone marrow or liver, but may also include the skin, brain, bone and other tissues.
Within each stage, the disease is categorized as either symptomatic or asymptomatic.
Types of Lymphoma
Dozens of related lymphoma types appear and act differently in the body. While lymphoma is a rare disease, some types are more common. About 90% of people with lymphoma are diagnosed with non-Hodgkin lymphoma. Hodgkin and non-Hodgkin tend to behave differently in the body, and treatments for each disease differ.
Non-Hodgkin lymphoma occurs when either B or T cell lymphocytes become abnormal. In most cases, it is the B cells that are defective. Non-Hodgkin lymphomas are categorized based on a spectrum of how quickly they develop in the body and how well they respond to treatment.
- Diffuse large B cell lymphoma, the most common type of non-Hodgkin lymphoma, is fast-growing cancer that tends to start in lymph nodes in the upper body, such as the chest, neck, abdomen or armpit.
- Follicular lymphoma, the second most common type of non-Hodgkin lymphoma, starts in lymph nodes throughout the body and the bone marrow. Over time, this typically slow-growing disease can become a more aggressive type.
- Small cell lymphocytic lymphoma (SLL) and chronic lymphocytic leukemia (CLL) are the same diseases found in different areas. SLL cells tend to be found in lymph nodes, while CLL cells are in the blood and bone marrow.
- Mantle cell lymphoma develops from cancerous cells in a lymph node's mantle zone (outer edge). It can be fast- or slow-growing and may spread to the gastrointestinal tract and bone marrow.
- Slow-growing marginal zone lymphomas account for about 5-10% of lymphomas. The cells in these lymphomas look small under the microscope. There are three main types of marginal zone lymphomas: mucosa-associated lymphoid tissue lymphoma, also called extranodal marginal zone lymphoma, develops outside the lymph nodes in organs like the stomach, thyroid, eyes, small intestine and lungs; nodal marginal zone lymphoma occurs within the lymph nodes; and splenic marginal zone lymphomas develop in the spleen and blood and are associated with hepatitis C infection.
- Burkitt lymphoma is one of the fastest-growing types affecting both adults and children. It is most often associated with Epstein-Barr virus infection but can occur in anyone with a weakened immune system.
- Waldenstrom macroglobulinemia begins when the body produces too much of a protein called immunoglobulin, which thickens the blood. As a result, Waldenstrom cells may crowd out normal cells in bone marrow, like red and white blood cells and platelets.
- Primary central nervous system lymphoma develops in lymph tissue located in the brain and spinal cord.
- Lymphoblastic lymphoma is a rare form of non-Hodgkin lymphoma involving abnormal B or T cells, most often affecting children.
- HIV- and AIDS-related lymphoma develops in patients who have acquired immune deficiency syndrome. Patients with weakened immune systems are at significantly greater risk of lymphoma.
- Cutaneous T cell lymphomas involve the skin and start with rash, intense itching, dry skin, pain and enlarged lymph nodes. The most common CTCLs include mycosis fungoides and Sezary syndrome.
- Anaplastic large-cell lymphoma is a rare type that can appear in lymph nodes, organs within the body or skin. Anaplastic large cell and cutaneous lymphomas are among several subtypes of peripheral T cell lymphoma.
- Composite lymphoma is a rare type composed of several different types of lymphoma cells.
Hodgkin lymphoma occurs when white blood cells called B-lymphocytes become abnormal and begin growing and dividing so fast that normal cells in the immune system cannot keep up. Hodgkin lymphoma cells are called Reed-Sternberg — large cells with more than one nucleus that resemble "owl's eyes." Hodgkin lymphoma is rarer than non-Hodgkin and is most common among young people.
How Lymphoma Develops
Lymphoma is not just one disease, but a large, complex group of blood cancers that start in a part of the immune system called the lymphatic system. This system of tissues, organs and vessels comprises pea-sized organs (called nodes) where white blood cells cluster, connected by thin tubes (called vessels). Because this system is widespread, cancers involving the lymphatic system can begin in almost any body part.
An Abnormal Immune Response
Lymphoma develops when lymphocytes — infection-fighting white blood cells in the immune system — become abnormal and grow and divide uncontrollably into tumors. Lymphocytes are the central part of the immune system and circulate throughout the body responding to bacteria and viruses.
With lymphoma, abnormal white blood cells grow in number (also causing the lymph nodes to swell) not because of an infection but because of a defect inside the cells. Instead of fighting disease, these cells, which have no real purpose in the body, grow and take up space. In contrast to a normal immune response, swelling in the lymph nodes does not subside with lymphoma.
What Increases Your Risk of Lymphoma?
Things that put you at higher risk for getting lymphoma are called risk factors. Doctors do not know what causes most lymphomas, and very little can be done to prevent them. For most patients, it is not one but a combination of factors that likely contributes to developing lymphoma.
This includes HIV/AIDS, other autoimmune diseases and being on immune-suppressing medication.
Age increases the risk of most cancers, including lymphoma.
Males are more likely than females to be diagnosed.
Caucasians are more likely to develop lymphoma.
Chemotherapy and radiation can increase risk of lymphoma.
Infections, such as the Epstein-Barr virus, human T cell leukemia virus type 1 and human herpes virus-8, increase risk of certain lymphoma subtypes. Other infections, such as chlamydia, helicobacter pylori and hepatitis C, are also associated.
Having a brother, sister or parent with lymphoma can slightly increase risk of certain lymphomas.
Exposure to chemicals, pesticides and other toxins, like Agent Orange and benzene, may be linked to developing lymphoma.
Being overweight is associated with developing several cancers and, in some studies, has shown an association with lymphoma.
Maintaining a healthy weight and diet has been shown in some studies to lower the risk of developing lymphoma. Studies have linked eating a diet rich in fruits and vegetables with substantially reduced risk of lymphoma and, as with many cancers, being physically active and not overconsuming products with animal fats may also help.
What Are the Symptoms of Lymphoma?
Usually, there are no early warning signs of lymphoma, so most diagnoses occur during later stages. Some of the first signs of lymphoma are the same as other illnesses, including swollen lymph nodes, cough and fever, making it challenging to diagnose early on. The most common symptoms of lymphoma include:
- Swollen lymph nodes (in the neck, armpits or groin) that do not go away
- Severe itching all over the body
- Excessive sweating, especially at night
- Unexplained weight loss
- Unusual tiredness or weakness that does not go away
- Abdominal discomfort, pain or fullness when eating (due to an enlarged spleen)
- Low red blood cell count
- Shortness of breath or persistent cough
- Chest pain or pressure
While swollen lymph nodes associated with lymphoma may first appear in any body part, swelling associated with Hodgkin lymphoma is more likely to start in the upper body, including the neck, chest and sometimes the abdomen.
Symptoms of other medical conditions may mirror those of lymphoma. Therefore, if you are treated for those conditions, or if your symptoms last for several weeks despite medical treatment, you may need further consultation to rule out lymphoma.
At City of Hope, our world-renowned hematopathologists perform leading-edge diagnostics to ensure the best outcomes for our lymphoma patients.