Once non-Hodgkin lymphoma is diagnosed, tests are done to determine the stage (extent of spread) of the disease. The treatment and prognosis (outlook) for a patient with non-Hodgkin lymphoma depend on both the exact type and the stage of the lymphoma.

Tests used to gather information for staging include:

• physical exam
• blood tests
• imaging tests, including a chest x-ray and CT or MRI scan of the chest/abdomen/pelvis (other tests such as a PET scan may also be used)
• bone marrow aspiration and biopsy (often but not always done)
• lumbar puncture (spinal tap -- this is not often done)

These tests are described in the section, "How is non-Hodgkin lymphoma diagnosed?"

Ann Arbor staging system

The staging system most often used to describe the extent of non-Hodgkin lymphoma in adults is called the Ann Arbor staging system. The stages are described by Roman numerals I through IV (1-4). Lymphomas that affect organs outside of the lymph system (extranodal organs) have E added to their stage (for example, stage IIE), while those affecting the spleen have an S added.

Stage I

If either of the following is present it means the disease is stage I:

• The lymphoma is in a lymph node or nodes in only 1 region, such as the neck, groin, underarm, and so on.

• The cancer is found only in 1 area of a single organ outside of the lymph system (IE).

Stage II

If either of the following is present it means the disease is stage II:

• The lymphoma is in 2 or more groups of lymph nodes on the same side of (above or below) the diaphragm (the muscle that separates the chest and abdomen). For example, this might include nodes in the underarm and neck area but not the combination of underarm and groin nodes.

• The lymphoma extends locally from a single group of lymph node(s) into a nearby organ (IIE). It may also affect other groups of lymph nodes on the same side of the diaphragm.

Stage III

If either of the following is present it means the disease is stage III:

• The lymphoma is found in lymph node areas on both sides of (above and below) the diaphragm.

• The cancer may also have spread into an area or organ next to the lymph nodes (IIIE), into the spleen (IIIS), or both (IIISE).

Stage IV

If either of the following is present it means the disease is stage IV:

• The lymphoma has spread outside of the lymph system into an organ that is not right next to an involved node.

• The lymphoma has spread to the bone marrow, liver, brain or spinal cord, or the pleura (thin lining of the lungs).

Along with the Roman numeral, each stage is also assigned an A or B. The letter B is added (stage IIIB, for example) if any of the following "B symptoms" are present:

• unexplained weight loss (more than 10% of weight)
• soaking night sweats
• unexplained fever of at least 101.5°F

These symptoms usually mean the disease is more advanced. If none of these B symptoms is present, the letter A is added to the stage.

The type and stage of the lymphoma provide useful information about a person's prognosis, but for some types of lymphomas (especially fast-growing ones) the stage is not too helpful on its own. In these cases, other factors can give doctors a better idea about a person's prognosis (outlook).

International Prognostic Index

The International Prognostic Index (IPI) was first developed to help determine the outlook for people with fast-growing lymphomas. However, it has proven useful for most other lymphomas as well (other than slow-growing follicular lymphomas, which are discussed below). The index depends on 5 factors:

• a person's age
• the stage of the lymphoma
• whether or not it is in organs outside the lymph system
• performance status (PS) -- how well a person can complete normal daily activities
• the blood (serum) level of lactate dehydrogenase (LDH) -- this level goes up in the presence of fast-growing tumors

Good prognostic factors	Poor prognostic factors
Age 60 or below	Age above 60
Stage I or II	Stage III or IV
No lymphoma outside of lymph nodes, or lymphoma in only 1 area outside of lymph nodes	Lymphoma is in more than 1 organ of the body outside of lymph nodes
PS: Able to function normally	PS: Needs a lot of help with daily activities
Serum LDH is normal	Serum LDH is elevated

Each poor prognostic factor is assigned 1 point. People without any poor prognostic factors would have a score of 0, while those with all of the poor prognostic factors would have a score of 5. The index divides people with lymphomas into 4 risk groups:

• low (0 or 1 poor prognostic factors)
• low intermediate (2 poor prognostic factors)
• high intermediate (3 poor prognostic factors)
• high (4 or 5 poor prognostic factors)

In the studies used to develop the index, about 75% of people in the lowest risk group lived longer than 5 years, whereas only about 30% of people in the highest group lived at least 5 years. These numbers show the difference the index scores can make, but newer treatments have been developed since then, so current survival rates are likely to be higher.

The IPI allows doctors to plan treatment better than they could just based on the type and stage of the lymphoma. This has become more important as new, more effective treatments have been developed that sometimes have more side effects. The index helps doctors figure out whether these treatments are needed. It also gives patients information about the outlook for their future.

Follicular Lymphoma International Prognostic Index

The IPI is useful for most lymphomas, but it is not as helpful for follicular lymphomas, which tend to be slower growing. Doctors have developed the Follicular Lymphoma International Prognostic Index (FLIPI) specifically for this type of lymphoma. It uses slightly different prognostic factors than the IPI.

Patients are assigned a point for each poor prognostic factor. People without any poor prognostic factors would have a score of 0, while those with all poor prognostic factors would have a score of 5. The index then divides people with follicular lymphoma into 3 groups:

• low risk: no or 1 poor prognostic factor(s)
• intermediate risk: 2 poor prognostic factors
• high risk: 3 or more poor prognostic factors

The study used to develop the FLIPI yielded the following survival rates:

These rates reflect the number of people who lived for at least 5 or 10 years after being diagnosed – many people lived longer than this.

Last Medical Review: 07/17/2009
Last Revised: 07/17/2009