The most significant B symptoms are fevers and weight loss. Night sweats alone do not confer an adverse prognosis. Pruritus as a systemic symptom remains controversial and is not considered a B symptom in the Ann Arbor staging system. (For more information on Pruritus, refer to the PDQ summary of the same name.) This symptom is hard to define quantitatively and uniformly, but when it is recurrent, generalized, and otherwise unexplained, and when it ebbs and flows parallel to disease activity, it may be the equivalent of a B symptom.
The designation E is used when well-localized extranodal lymphoid malignancies arise in or extend to tissues beyond, but near, the major lymphatic aggregates. Stage IV refers to disease that is diffusely spread throughout an extranodal site, such as the liver. 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.
Notations for Identifying Sites
|N = nodes
||H = liver
||L = lung
|| M = bone marrow
|S = spleen
|| P = pleura
||O = bone
|| D = skin
Current practice is to assign a clinical stage (CS) based on the findings of the clinical evaluation and a pathologic stage (PS) based on the findings of invasive procedures.
For example, a patient who has disease in the chest and neck, systemic symptoms, and a negative lymphangiogram might be found at laparotomy to have involvement of the spleen, liver, and bone marrow. Thus, the precise stage of such a patient would be CS IIB, PS IVB (S+)(H+)(M+).
Stage I adult HL is characterized by the involvement of a single lymphnode region (I) or localized involvement of a single extralymphatic organ or site (IE)
Stage II adult HL is characterized by the 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 node(s) with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript
Stage III adult HL is characterized by the involvement of lymph node regions on both sides of the diaphragm (III), which may also be accompanied by localized involvement of an associated extralymphatic organ or site (IIIE), by involvement of the spleen (IIIS), or by involvement of both (IIIE + S). Stage III disease may be subdivided by anatomic distribution of abdominal involvement or by extent of splenic involvement. Stage III (1) indicates involvement that is limited to the upper abdomen above the renal vein. Stage III (2) indicates involvement of pelvic and/or para-aortic nodes. Five or more visible splenic nodules on a cut section constitutes extensive splenic involvement. Zero to four nodules is classified as minimal splenic disease.