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Detailed Guide: Lymphoma, Non-Hodgkin Type
How Is Non-Hodgkin Lymphoma Staged?
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 spread 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 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 aids breathing and 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 extended into an area or organ next to the lymph nodes (IIIE), into the spleen (IIIS), or both (IIIE,S).


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 A is added if the person doesn't have any symptoms of lymphoma. The letter B is added (stage IIIB, for example) if any of the following symptoms are present:

  • unexplained weight loss (more than 10% of weight) 
  • soaking night sweats 
  • unexplained fever > 100°

Although the type and stage of the lymphoma provide useful information about a person's prognosis, for some types of lymphomas (especially fast-growing ones) the stage is not too helpful on its own. Other factors are looked at to help overcome this.

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 
  • a person's performance status (PS) -- how well the 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 present 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 5 years or longer. While these numbers show the difference the index scores can make, newer treatments have been developed since then, so current survival rates are likely to be higher.

This prognostic index is important because it allows doctors to plan treatment better than they could from just the pathology report and staging information. 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

Although the IPI is useful for most lymphomas, 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.

Good Prognostic Factors

Poor Prognostic Factors

Age 60 or below Age above 60
Stage I or II Stage III or IV
Blood hemoglobin 12 g/dL or above Blood hemoglobin level below 12 g/dL
Four or fewer lymph node areas affected More than four lymph node areas affected
Serum LDH is normal Serum LDH is elevated

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 included a group of about 1,800 patients and was reported in 2004. It yielded the following survival rates:


Risk Group
5-year Survival Rate
10-year survival rate
low-risk
91%
71%
intermediate-risk
78%
51%
high-risk
53%
36%


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: 08/29/2007
Last Revised: 05/12/2009

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