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1578 Part XI: Malignant Lymphoid Diseases Chapter 95: General Considerations for Lymphomas 1579

Although marrow aspiration and biopsy have been standard in TABLE 95–4. The Lugano Staging System for
lymphoma staging in the past, the high sensitivity of PET/CT for mar- 17
row involvement has rendered this procedure unnecessary for patients Lymphomas
with HL and DLBCL who have negative PET/CT imaging of the bones Stage* Involvement † Extranodal (E)
and marrow. Data for other histologies are currently insufficient and Status
a single 2.5-cm core biopsy with flow cytometry and cytogenetics are LIMITED
still recommended for full staging of other subtypes. 16,17 Standard blood I One nodal group Single extranodal
testing should also be performed, including a complete blood count involved lesions without
(CBC) and chemistries. Lactate dehydrogenase and β -microglobulin nodal involvement
2
are important serum prognostic markers that should be assessed at
baseline for most lymphomas. 33,146 Table 95–3 lists the staging proce- II Two or more nodal Stage I or II nodal
dures that are currently recommended and the criteria used to assign a groups involved, on involvement with
the same side of the limited, contig-
patient’s stage (Table 95–4). 17 diaphragm uous extranodal
At the completion of therapy, all diagnostic studies performed at extension
baseline detecting evidence of disease are repeated for response eval- ‡
uation. Current recommendations suggest that PET/CT imaging be II bulky As in II above, but Not applicable
interpreted using visual inspection according to a 5-point “Deauville with “bulky” disease
15
scale” (Table 95–5). Deauville scores of 1 to 2 on FDG-PET scans ADVANCED
indicate metabolic activity in tumor sites less than in the mediasti- III Involvement of Not applicable
nal blood pool, signifying complete metabolic response and complete nodal groups on
remission. In contrast, Deauville scores of 4 or 5 at the end of treatment, both sides of the
diaphragm §
IV Diffuse involvement Not applicable
TABLE 95–3. Staging Procedures for Lymphoma of a visceral organ
not contiguous with
Initial studies an involved nodal
History and physical examination site
CBC
Metabolic panel including renal and hepatic function CT, computed tomography; DLBCL, diffuse large B-cell lymphoma;
FDG, 2-fluorodeoxyglucose; HL, Hodgkin lymphoma; NHL, non-
Uric acid Hodgkin lymphoma; PET, positron emission tomography.
Lactate dehydrogenase and/or β -microglobulin *Stages are refined further for patients with HL by designating
2
Hepatitis B and C serologies (if rituximab therapy planned) whether or not “B symptoms” are present, namely, fevers greater than
HIV serology 38.3°C, drenching night sweats, or unexplained weight loss of more
than 10% of body mass over 6 months. Current recommendations
Tumor biopsy specimen with histopathology discourage applying A and B designations to staging for patients
Flow cytometry of tumor specimen with NHL because these features do not confer independent prog-
17
Immunohistochemistry of tumor specimen nostic information.
† Extent of disease is assessed by PET/CT imaging for FDG-avid lym-
Cytogenetic analysis (including iFISH for lymphoma-associated phomas and by CT imaging for nonavid histologies.
translocations)
‡ A nodal mass of ≥10 cm, or greater than one-third of the transtho-
PET/CT scans of neck, chest, abdomen, and pelvis (for FDG-avid racic diameter at any level of thoracic vertebrae as determined by CT
lymphomas) imaging is considered bulky disease for HL. There is no consensus on
Contrast-enhanced CT scans of neck, chest, abdomen, and pel- the size of “bulk” for NHL with a suggestion that 6 cm may be optimal
vis (particularly for lymphomas that are not FDG-avid) for follicular lymphoma. Sizes between 6 cm and 10 cm have been
17
Additional studies (useful in selected cases) advocated to define bulk for DLBCL. Current recommendations are
Marrow aspiration and biopsy to record the longest measurement by CT scan and not employ the
“X” notation to designate bulky disease. Stage II bulky disease may be
Pregnancy testing in women of childbearing potential considered to be either limited or advanced disease depending on
Immunoglobulin and TCR gene rearrangement studies histology and associated prognostic factors.
Cardiac ejection fraction measurement (if anthracycline therapy § Tonsils, Waldeyer ring, and spleen are considered nodal tissue in this
planned) staging system.
Magnetic resonance imaging of brain if neurologic signs or
symptoms
Cerebrospinal fluid analysis (including flow cytometry) for high- indicate residual abnormal metabolic activity, representing treatment
risk aggressive lymphomas or if neurologic signs or symptoms failure (Table 95–6). A Deauville score of 3, indicating metabolic activ-
are present ity greater than the mediastinum but less than the liver, is indetermi-
Gastrointestinal studies (imaging and endoscopy) if Waldeyer nant. Most patients with HL or DLBCL who have a Deauville score of
ring involvement, mantle cell lymphoma, or enteropathy asso- 3 at the end of treatment have good outcomes, but careful followup of
ciated lymphoma
such patients is important.
CBC, complete blood count; CT, computed tomography; FDG, The International Working Group (IWG) and the National Can-
2-fluorodeoxyglucose; iFISH, interphase fluorescence in situ hybrid- cer Center Network (NCCN) have published recommendations for fol-
ization; PET, positron emission tomography; TCR, T-cell receptor. lowup of patients in remission that vary by histology, whether a patient

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