Stage 1 Involvement of a single lymph node region or a single extralymphatic site (IE) Stage 2 Involvement of two or more lymph node regions on the same side of the diaphragm, may have contiguous extralymphatic tissue involvement (IIE) Stage 3 Involvement of lymph node regions on both sides of the diaphragm, possibly with spleen involvement (IIIS) or extralymphatic involvement (IIIE) Stage 4 Diffuse extralymphatic involvement
A or B B refers to the presence of systemic symptoms including fevers, night sweats, and significant weight loss (>10% body weight over 6 mo)
without fixative, promptly to the pathologist. Drying creates artifact that compromises an accurate reading of the histopathology.
Following tissue diagnosis, characterization of the extent of disease, or staging, is performed. The Ann Arbor staging system (Table 2) (37), originally devised for Hodgkin's disease, is also applied to NHL. The TNM system (tumor, nodes, and metastases) is not used for lymphomas, primarily due to the difficulty in specifying primary versus secondary sites. Additionally, the histology of the lymphoma is just as important as the anatomic distribution in dictating management and outcome. A bone marrow evaluation should be performed in all cases of lymphoma, with the exception of some very limited-stage cases of Hodgkin's disease. Computed tomography of the chest, abdomen, and pelvis completes the formal staging procedures. Increasingly, positron-emission tomography (PET scan) is performed because it can provide useful staging information at diagnosis or relapse and prognostic information during or following treatment. PET scans, when obtained pre- and post-treatment, allow differentiation of residual active disease from lymphadenopathy due to fibrosis (38).
A complete blood count and serum chemistry profile, including liver enzymes and serum lactate dehydrogenase (LDH), should also be obtained. Hepatic lymphoma involvement may be suggested by the finding of elevated transaminases, even in the setting of a normal-appearing liver. If dealing with a lymphoplasmacytic lymphoma and the possibility of WM, a serum protein electrophoresis, immunofixation, quantitative Igs, and serum viscosity should be obtained. The immunofixation will allow identification of the monoclonal protein as IgM. Most cases of MM are monoclonal IgG, with IgA and IgD occurring less frequently. Monoclonal IgG is also the most common paraprotein elaborated in plasmacytomas, although the non-IgG types are more likely to progress later to MM. Solitary plasmacytomas are diagnosed after excluding anemia and hypercalcemia. If present, a monoclonal paraprotein should resolve completely following therapy. Soft-tissue solitary plasmacytomas are much less likely to progress to MM, with approximately 70% remaining disease-free at 10 years (39).
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