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
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 lymph node
region (I) or localized involvement of a single extralymphatic organ or site
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.
Stage IV adult HL is characterized by 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.
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
Many investigators and many new clinical trials employ a clinical staging system that divides patients into three 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 CXR, =10 cm on CT scan).
- Extranodal involvement.
- Elevated ESR (>30 mm/hr for B stage, >50 mm/hr for A stage).
- Three or more lymph node areas' involvement.
- B symptoms.
- ADVANCED: Clinical stage III or IV. 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 seven adverse factors:
- Albumin level of less than 4.0 g/dL.
- Hemoglobin level of less than 10.5 g/dL.
- Male sex.
- Age of 45 years or older.
- Stage IV disease.
- White blood cell
(WBC) count of at least 15,000/mm3.
- Absolute lymphocytic
count of less than 600/mm3 or a lymphocyte count that was less than 8% of the total WBC count.