Non-Hodgkin's Lymphoma

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Questions to Ask Your Doctor About Your Cancer
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Staging

Once the physician has diagnosed lymphoma, he or she will want to perform studies to establish the patient's stage - that is, to find out how far the patient's cancer has spread. Staging helps the physician to select appropriate treatment options and helps him/her to arrive at a prognosis, or estimate of disease outlook and survival. Clinical information is reviewed, including findings from the physical examination, blood tests, and imaging studies. The imaging studies most often employed are chest X-ray and computed tomographic (CT) scan of the chest, abdomen, and pelvis.

If the patient has non-Hodgkin's lymphoma (NHL), the physician may order additional tests such as blood tests that reflect kidney and liver function which are important factors in the choice of chemotherapeutic drugs, bone marrow aspiration/ biopsy, and lumbar puncture.

Lumbar puncture, otherwise known as a "spinal tap," is performed if the physician suspects that NHL has spread to the central nervous system (CNS) or bone marrow. In addition, lumbar puncture is performed routinely for certain aggressive lymphomas (e.g., primary central nervous system lymphoma, or PCNSL, in AIDS patients).

The Ann Arbor Staging
As with many other cancers, NHL is categorized on the basis of tumor burden. The Ann Arbor Staging System is the most popular system for classifying NHL. The Ann Arbor Staging groups are as follows:

After an NHL patient has been assigned a stage, this categorization may be refined by adding the biologic grade of the disease, that is, "low," "intermediate," or "high" grade. Other descriptive terms - such as "bulky" versus "non-bulky" disease and the presence or absence of B symptoms - may be used to fully describe a particular case of lymphoma.

Additional Designations (applicable to any of the stages of HD or NHL):

• A - absent (no) symptoms.
• B - Presence of any of the following B symptoms: fever (greater than 101.5°), drenching night sweats, unexplained weight loss of 10% or more within the last 6 months, severe itching (see Signs & Symptoms of Lymphoma).
• E -involvement of a single extranodal (other than the lymph nodes) site that directly adjoins or is next to the known nodal group.
• X - Presence of "bulky" disease, that is, a nodal mass whose greatest dimension is more than 10 centimeters in size, and/or
  a widening of the mediastinum (middle chest) by more than one-third.
• CS - clinical stage as obtained by doctor's examinations and tests.
• PS - pathological stage as obtained by exploratory laparotomy (surgery performed through an abdominal incision) with splenectomy (surgical removal of the spleen).

Example: Stage 2BX, high-grade NHL = NHL in which the disease is limited to two lymph node groups on the same side of the diaphragm (e.g., neck, underarm); the lymphoma is high-grade, with bulky disease and the presence of B symptoms.

International Prognostic Index
The International Prognostic Index (IPI) was designed to further clarify lymphoma staging. The IPI predicts the risk of disease recurrence and overall survival by taking into account factors such as age, stage of disease, general health (also known as performance status), number of extranodal (other than the lymph nodes) sites, and the presence or absence of an elevated serum enzyme named lactate dehydrogenase (LDH).

Example: A patient with the following prognostic factors would have an IPI score of 5, making him or her a "high risk" patient. The individual would have a 40% chance of being relapse-free over the next 5 years, a 44% chance of complete response, and a 26% chance of surviving for 5 years.

• Age over 60 years
• Late-stage disease (Stages 3 and 4)
• More than one extranodal site
• High LDH
• Poor general health

Table 3: International Prognostic Index Scoring

Please remember that these numbers are based on a group of individuals with the same qualities. However, you are an individual and may do better or worse than the above percentages. The chart is only to help your physician gain additional insight into your particular condition so appropriate recommendation can be made based on potential risk.

The IPI is used to identify patients who are at low risk for disease recurrence with standard therapy. In addition, the IPI can identify individuals who are at high risk for disease recurrence despite receiving standard therapy. In such cases, the physician may suggest appropriate clinical trials for treatment.

Finally, the IPI can sort out intermediate risk patients who should be counseled about the options of conventional versus more aggressive therapy.

NHL Staging in Children
Several different systems are available for the staging of NHL in children (e.g., the St. Jude Children's Research Hospital system, the Children's Cancer Group system, etc.). Perhaps the most popular system is the St. Jude Children's Research Hospital model, which separates patients on the basis of limited versus extensive disease.

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