Non-Hodgkin’s lymphoma is a cancer of the immune system that specifically forms in the lymphatic tissues. When cancer develops in these tissues, it begins in a specific type of white blood cell called a lymphocyte.
There are both Hodgkin’s and non-Hodgkin’s lymphoma (NHL), but NHL is more common. Once a doctor confirms your NHL diagnosis, your treatment and outlook will depend on the specific type of NHL you have and the stage (or progression) of the disease.
This article will explore the different stages of non-Hodgkin’s lymphoma, how doctors stage NHL, and what stages mean to your treatment and outlook.
Why names matter
Once you’re diagnosed with NHL, it’s not as simple as having a name for your condition. You will undergo more testing to find out the specific type of non-Hodgkin’s lymphoma that you have. Your treatment and outlook are linked to the type and stage of your lymphoma.
Staging is an important tool in diagnosing and treating most types of cancer. It helps your doctor understand how far your cancer has spread and how advanced the disease is.
Generally, the first two stages of NHL are considered early or limited stages. The second two are considered advanced stages.
This is the earliest stage that a doctor will diagnose in NHL. You have stage 1 NHL if your doctor finds cancer cells in only one lymph node area or one lymphatic organ like the tonsils or spleen.
If cancer cells are only in one area, but it’s in an organ outside of the lymphatic system, NHL is classified as Stage 1E. The “E” stands for “extranodal,” or outside the lymph nodes.
Another early stage of lymphoma, stage 2 NHL, is diagnosed when you have cancer cells in two or more groups of lymph nodes either above or below your diaphragm, but not both. For example, lymphoma cells found in the lymph nodes in the underarm and the neck would be classified as stage 2. If there are cancer cells in one area above the diaphragm and one below the diaphragm — for example, underarms and groin — it’s considered to be the more advanced stage 3.
Another stage 2 diagnosis — stage 2E — occurs when doctors find a group of cancer cells in a nearby organ.
As mentioned above, you’re diagnosed as stage 3 if lymphoma cells are in lymph nodes both above and below the diaphragm. You may also be considered to have stage 3 NHL if you have cancerous cells in a lymph node above the diaphragm, as well as in the spleen.
The most advanced stage of NHL, stage 4, is diagnosed when you have widespread lymphoma cells in an organ outside of the lymphatic system.
In some cases, doctors might use other terms to describe your NHL. These terms are not necessarily stages but characteristics of your disease that can impact your outlook or treatment.
- Bulky disease. Bulky disease describes cases of NHL where large lymphomas develop. Whether a doctor considers a lymphoma “bulky” or not often depends on the type of NHL and the doctor’s own judgment. Patients with bulky lymphomas require more intensive treatment.
- E (extranodal). Doctors use the suffix ‘E’ to refer to an NHL diagnosis where cancer cells are located outside of the lymph nodes. For example, stage 1 occurs in only one area of a lymph node or lymphatic organ. Stage 1E, however, specifies that while cancer cells are only in one area, they’re located in an organ that is outside of the lymph nodes or lymphatic system.
- Other suffixes. In the past, other suffixes — like A, B, S, and X — were also used to describe specific subtypes of NHL. For example, ‘S’ represented NHL that was found in the spleen, and ‘X’ indicated bulky disease. These designations are now made within the stages themselves, and doctors no longer use these suffixes.
Staging for non-Hodgkin’s lymphoma was first developed more than 60 years ago, but the system has undergone many updates as the understanding of NHL and disease detection have improved.
Doctors used the Cotswolds-modified Ann Arbor system until around 2011. At that time, significant updates — like removing additional suffixes — were made to the guidelines. The new staging system, adopted in 2011, is known as the Lugano staging classification. It is now the most commonly used classification tool in clinical practice.
Staging NHL involves extensive physical exams and tests. First, your doctor will ask you about (or examine you for) symptoms like:
- night sweats
- weight loss (10 percent or more over 6 months)
- a history of other cancers
- enlarged lymph nodes
- spleen enlargement or liver enlargement
Some of these can be identified through a physical examination and by taking your personal medical history. In many cases, though, additional testing will be used to confirm your diagnosis and assess staging.
Doctors perform most staging using positron emission tomography (PET) or computed tomography (CT) scans. But they may use magnetic resonance imaging (MRI) if they suspect NHL has spread to the nervous system.
Biopsies, when doctors remove samples of tissue for testing, are usually required when NHL has developed outside the lymph nodes or lymphatic system. They might do this for areas like the:
- gastrointestinal tract
- bone marrow
Knowing the stage of your NHL will help you and your doctor understand the severity and spread of your disease. Stages 1 and 2 are earlier stages, but cancer that has reached stages 3 or 4 — or that has a bulky designation — is more advanced and may be more difficult to treat.
There are many different types of NHL, and treatment will depend not only on the stage but also on the type of lymphoma. About
You’ll receive these medications for three to six cycles, with each cycle spaced about 3 weeks apart.
For DLBCL in stages 3 and 4, the first step of treatment is usually six cycles of R-CHOP, followed by more imaging and tests to evaluate how well the chemotherapy worked. You may need additional R-CHOP cycles or alternative regimens, as well as other therapies.
Follicular lymphoma (FL) is the second most common type of NHL. Doctors treat this differently, but it also depends on the stage.
But most people with FL have stage 3 or 4 when diagnosed. For advanced stages, treatment usually involves R-CHOP or a similar combination of drugs.
For NHL that is difficult to treat or comes back after treatment, advanced treatments might include targeted therapy (like the direct injection of chemotherapy medications into the spinal fluid) or stem cell transplants. Your doctor may also suggest that you enroll in clinical trials.
Overall, the 5-year survival rate for non-Hodgkin’s lymphoma is 73 percent. The outlook varies by stage and any subtype of the diagnosis.
- Stage 1: 84 percent
- Stage 2: 77 percent
- Stage 3: 71 percent
- Stage 4: 64 percent
How quickly you are diagnosed and move into treatment can impact your survival rate and overall outlook. Other factors also play a role in how NHL develops and how well your body is able to fight it. Some risk factors for NHL include:
- age over 60
- male gender
- immune system deficiencies
- autoimmune disorders
- previous cancer treatments
- exposure to chemicals or other toxins
Early symptoms that can raise concerns for NHL include:
- enlarged lymph nodes
- feeling full after not eating much
- weight loss
- chest pain or pressure
- shortness of breath
- swollen abdomen
- frequent or severe infections
- easy bruising or bleeding
Talk to your doctor if you have these symptoms or other types of problems that keep coming back or never go away. Some cancers, especially NHL, can appear with “B symptoms” like fever or night sweats.
Regular visits with your primary care doctor can allow you to discuss any health concerns and catch conditions like NHL early. Early diagnosis and treatment are the best tools for fighting cancers, as more advanced disease is usually more difficult to treat.
Fighting NHL and other cancers can be difficult for both you and your family. Talk to your doctor about resources in your community that you can go to for