Staging Subclassification System

Although stage is important in selecting the treatment of patients with non-Hodgkin lymphoma (NHL) who do not have acquired immunodeficiency syndrome (AIDS), the majority of patients with AIDS-related lymphomas have far-advanced disease. In general, the staging system used is the Ann Arbor system, which is identical to that used for non-AIDS-related NHLs.

Stages I, II, III, and IV NHL can be subclassified into A and B categories: B for those with well-defined generalized symptoms and A for those without. The B designation is given to patients with any of the following symptoms:

• Unexplained loss of more than 10% of body weight in the 6 months before diagnosis.
• Unexplained fever with temperatures higher than 38° C.
• Drenching night sweats. (Refer to the PDQ summary on Fever, Sweats, and Hot Flashes for more information.)

The designation “E” is used when extranodal lymphoid malignancies arise in tissues away from the major lymphatic aggregates. If pathologic proof of involvement of one or more extralymphatic sites has been documented, the symbol for the site of involvement, followed by a plus sign (+), is listed. Sites are identified by the following notation:

Stage I

Stage I NHL means involvement of a single lymph node region (I), or localized involvement of a single extralymphatic organ or site (IE).[1,2]

Stage II

Stage II NHL means involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE).[1,2]

Stage III

Stage III NHL means involvement of lymph node regions on both sides of the diaphragm (III) that may also be accompanied by localized involvement of an extralymphatic organ or site (IIIE), involvement of the spleen (IIIS), or both (IIIS+E).[1,2]

Stage IV

Stage IV NHL means disseminated (multifocal) involvement of one or more extralymphatic organs with or without associated lymph node involvement or isolated extralymphatic organ involvement with distant (nonregional) nodal involvement.[1,2]

A number of factors that are important for determining prognosis are not included in the staging system for NHLs. All of these factors should be considered when selecting treatment. Prognosis is related to the severity of the underlying immune deficiency (CD4 lymphocyte count), the presence or history of opportunistic infections (prior AIDS-defining illness), bone marrow involvement, performance status, and presence of extranodal disease.[3] Typically, AIDS-related lymphomas are widespread with extranodal disease at the time of presentation. The most common extranodal sites are the gastrointestinal (GI) tract, central nervous system, bone marrow, and liver. In one series, the largest group of patients had both extranodal and nodal disease (43%), but 33% of the patients presented with extranodal disease only.[4] In a second series, 87% of the patients had extranodal disease at presentation.[5]

At diagnosis, 66% of the patients have stage IV disease. In addition, unusual presentations include involvement of the rectum, heart, pericardium, pulmonary parenchyma, bile ducts, mouth, and subcutaneous and soft tissues. The clinical features of AIDS-related lymphomas correlate with histopathology. The majority of patients with small noncleaved cell (Burkitt) lymphomas present with stage IV disease, mostly because of bone marrow involvement. This compares with an approximately 40% stage IV presentation by those with immunoblastic and large cell lymphomas. A particular prevalence for GI involvement has been noted in patients who have immunoblastic and large noncleaved cell lymphoma types.[6] While high-risk behavior should be looked for in every patient, HIV testing should probably be done for any patient who has Burkitt lymphoma or the atypical presentation of extranodal lymphoma that involves rare sites, i.e., rectum, GI tract, bone, or orbit. Similarly, malignant lymphoma should be considered in any HIV-infected patient who has progressive lymphadenopathy, tumors at any site, central nervous system symptoms, or unexplained wasting, fever, or abdominal pain.

References

1. Lymphoid neoplasms. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 393-406. 
   
   
2. National Cancer Institute sponsored study of classifications of non-Hodgkin's lymphomas: summary and description of a working formulation for clinical usage. The Non-Hodgkin's Lymphoma Pathologic Classification Project. Cancer 49 (10): 2112-35, 1982.  [PUBMED Abstract]
   
   
3. Levine AM: Acquired immunodeficiency syndrome-related lymphoma: clinical aspects. Semin Oncol 27 (4): 442-53, 2000.  [PUBMED Abstract]
   
   
4. Kaplan LD, Abrams DI, Feigal E, et al.: AIDS-associated non-Hodgkin's lymphoma in San Francisco. JAMA 261 (5): 719-24, 1989.  [PUBMED Abstract]
   
   
5. Knowles DM, Chamulak GA, Subar M, et al.: Lymphoid neoplasia associated with the acquired immunodeficiency syndrome (AIDS). The New York University Medical Center experience with 105 patients (1981-1986). Ann Intern Med 108 (5): 744-53, 1988.  [PUBMED Abstract]
   
   
6. Raphael BG, Knowles DM: Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphoma. Semin Oncol 17 (3): 361-6, 1990.  [PUBMED Abstract]
   
   

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