Stage Information
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.
Staging Subclassification System
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:
Notation for identification of sites
N = nodes |
H = liver |
L = lung |
M = marrow |
S = spleen |
P = pleura |
O = bone |
D = skin |
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
-
Lymphoid neoplasms. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 393-406.
-
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]
-
Levine AM: Acquired immunodeficiency syndrome-related lymphoma: clinical aspects. Semin Oncol 27 (4): 442-53, 2000.
[PUBMED Abstract]
-
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]
-
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]
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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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