a Reprinted with permission from AJCC: Hodgkin and non-Hodgkin lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 607-11.
Involvement of a single lymphatic site (i.e., nodal region, Waldeyer ring, thymus or spleen) (I).
Localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE) (rare in Hodgkin lymphoma).
Involvement of ≥2 lymph node regions on the same side of the diaphragm (II).
Localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).
The number of regions involved may be indicated by an arabic numeral, as in, for example, II3.
Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE, S).
Splenic involvement is designated by the letter S.
Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement.
Isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s).
Stage IV includes any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.
Massive mediastinal disease has been defined by the Cotswolds meeting as a thoracic ratio of maximum transverse mass diameter of 33% or more of the internal transverse thoracic diameter measured at the T5/6 intervertebral disc level on chest radiography. Some investigators have designated a lymph node mass measuring 10 cm or more in greatest dimension as massive disease. Other investigators use a measurement of the maximum width of the mediastinal mass divided by the maximum intrathoracic diameter.
Many investigators and many new clinical trials employ a clinical staging system that divides patients into four major groups that are also useful for the practicing physician:
Early favorable: Clinical stage I or II without any risk factors.
Early unfavorable: Clinical stage I or II with one or more of the following risk factors:
Large mediastinal mass (>33% of the thoracic width on the chest x-ray, ≥10 cm on CT scan).
Elevated erythrocyte sedimentation rate (>30 mm/h for B stage, >50 mm/h for A stage).
Three or more lymph node areas' involvement.
Advanced favorable: Clinical stage III or IV with zero to three adverse risk factors listed below. Patients with advanced favorable disease have a 60% to 80% freedom-from-progression at 5 years from treatment with first-line chemotherapy.[Level of evidence: 3iiiDiii]
Advanced unfavorable: Clinical stage III or IV with four or more adverse risk factors listed below. Patients with advanced unfavorable disease showed a 42% to 51% freedom-from-progression at 5 years from treatment with first-line chemotherapy.[Level of evidence: 3iiiDiii]. For patients with advanced-stage HL, the International Prognostic Factors Project has developed an International Prognostic Index with a prognostic score that is based on the following seven adverse factors:
Absolute lymphocytic count of <600/mm3 or a lymphocyte count that was <8% of the total WBC count.
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