When you receive a diagnosis of follicular lymphoma, your doctor will usually determine the grade and stage of your cancer. Follicular lymphoma is one of the most common types of non-Hodgkin lymphoma, a cancer that affects your lymphatic system. Follicular lymphoma develops from B cells, a type of white blood cell important in the immune system for fighting off germs. FL gets its name from the follicles, or clumps of B cells, that form inside your lymph nodes.
The cancer’s grade provides information about how your B cells appear when examined under a microscope. The stage explains where in your body the lymphoma is located and how far it has spread. Furthermore, your doctor may use a prognostic score to help predict your outlook.
To determine the grade of your follicular lymphoma, a doctor will perform a lymph node biopsy, in which all or part of a lymph node is removed. Samples of the lymph node are carefully studied under a microscope. The doctor will count the number of cancerous cells, some of which may appear larger than the other healthy B cells. The doctor will then assign your lymphoma a grade depending on how many large cells the doctor sees and how they are arranged.
Follicular lymphomas may receive a grade of 1, 2, 3A, or 3B. Grade 1 lymphoma is at an earlier stage and has not produced many large cells. A lymphoma that is grade 3B is more advanced and has already made many large cancer cells.
Most cases of follicular lymphoma are initially diagnosed at a lower grade (grades 1, 2, or 3A). These lymphomas usually grow slowly and don’t cause many symptoms. Grade 3B, on the other hand, is considered high-grade lymphoma. It grows quickly and may require more aggressive treatment.
In some cases, follicular lymphoma may transform into a different type of lymphoma that is high-grade and faster-growing. It may develop into diffuse large B-cell lymphoma (DLBL), Burkitt’s lymphoma, high-grade B-cell lymphoma, or B-cell acute lymphoblastic leukemia (B-ALL).
To see where cancer cells are located and whether they have spread, your doctor will usually use imaging tests, such as a computerized tomography (CT) scan or positron emission tomography (PET) scan. These tests help your doctor visualize how cancer has spread so they can assign a stage to your lymphoma. You may also need to have a bone marrow biopsy to determine whether there are cancer cells in your bone marrow, where blood cells develop.
A follicular lymphoma stage is based on how many lymph nodes are affected, where these lymph nodes are located, and whether cancer cells can be found in other organs inside or outside of the lymphatic system. The higher the stage, the more advanced the lymphoma.
Your lymphoma stage can help your doctor predict your outlook and determine which treatment plan may be the best option.
Stage 1 lymphoma indicates early disease that has not yet spread. It only affects a single group of lymph nodes, or a single organ outside of the lymphatic system. Stage 1 lymphoma is slow-growing and may cause very few symptoms — or none at all. The symptom that often develops first is a swollen lymph node, which may appear as a lump in your armpit, neck, or groin.
If your lymphoma is at stage 1, you may receive radiation therapy. Stage 1 follicular lymphoma is often treated with radiation if the lymphoma is grade 1, 2, or 3A. If your lymphoma is grade 3B, it may require more aggressive treatment, including chemotherapy.
If your stage 1 follicular lymphoma consists of a small-sized tumor, radiation may be enough to completely eliminate the cancer. In one study, 40 people with stage 1 or 2, grade 1 or 2 follicular lymphoma were treated with radiation. Most of the trial participants had good outcomes, and after five years, two-thirds of participants remained disease-free.
In stage 2, lymphoma has begun to spread, but only to nearby areas. In this stage, lymphoma cells can be found in two different groups of lymph nodes located on the same side of the diaphragm — the muscle that separates your chest from your abdomen. Alternatively, a stage 2 follicular lymphoma diagnosis can also mean that cancer cells are found in one organ plus a nearby group of lymph nodes.
The need for lymphoma treatment at stages 2, 3, and 4 will depend on the person’s symptoms. People without symptoms may be managed with monitoring only, following a watchful waiting approach, where a person has frequent checkups to look for signs that the cancer is progressing. If someone has symptoms, they may receive treatments that aim to relieve those specific symptoms.
Like stage 1 lymphoma, stage 2 lymphoma may be curable with radiation treatments if the tumor is small in size.
In stage 3 follicular lymphoma, cancer cells have spread further, and lymphoma can be found on both sides of the diaphragm. Some people with stage 3 disease also have cancer in the spleen or other organs. Most cases of follicular lymphoma are diagnosed at advanced stages — stages 3 and 4.
The goal of first-line treatment is to eliminate the cancer. Maintenance treatment aims to get rid of remaining cancer cells and keep them from growing. First-line treatment often consists of chemotherapy, such as bendamustine (Treanda) or a combination of chemotherapy drugs that are referred to using the acronym CHOP. CHOP includes cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), vincristine (formerly sold as Oncovin), and prednisone. Other common induction treatments include the monoclonal antibody therapies rituximab (Rituxan) or obinutuzumab (Gazyva), which can specifically target and kill B cells.
Maintenance treatment often includes the use of rituximab. People following this treatment plan typically take this medication once every few months for up to two years.
Stage 4 is the most advanced stage of lymphoma, involving cancer that has spread to multiple organs or regions of the body. If someone has cancer cells in the bone marrow or in the liver, they will be diagnosed with stage 4 lymphoma.
As lymphoma spreads to other regions of the body, it may be more likely to cause symptoms. If you have enlarged lymph nodes next to an organ, the tumor can press on that organ and generate additional symptoms. For example, a tumor that is compressing the lungs may lead to coughing or breathing problems. Additionally, when lymphoma reaches the spleen or bone marrow, it often causes anemia, or low levels of red blood cells. Symptoms of anemia include fatigue, frequent infections, or bleeding problems.
Read more about how follicular lymphoma develops and common FL symptoms.
Stage 4 tumors are often treated using a similar plan as stage 3 lymphomas. First-line treatments with chemotherapy and rituximab may be followed by taking rituximab alone for a long period of time.
In addition to a number (1 through 4), your stage may also include the letter A or B. For example, your diagnosis may be stage 2A or 2B. These letters indicate whether you have symptoms. People with B stage lymphoma have a specific set of symptoms known as “B symptoms,” which may include fever, night sweats, or weight loss. People with A stage lymphoma don’t have these symptoms.
Other letters may also be added to the stage based on what parts of the body are affected. If there are cancer cells in any organs outside the lymph nodes, an E (which stands for extranodal) may be added to the stage. If there are cancer cells in the spleen, an S may be added.
Some doctors will use a tool such as the Follicular Lymphoma International Prognostic Index (FLIPI) to obtain a prognostic score between 0 and 5. This score estimates your outlook. This score is based on prognostic factors that help predict whether your cancer may get worse. These factors include:
Read more about how relapsed and refractory follicular lymphoma is treated.
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