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Staging of Hodgkin's lymphoma

The Ann Arbor staging system (1971) is used in a modified form, and is used by all hospitals in the Nordic countries. Primary extranodal lymphoma is a separate group and is abbreviated with Pe and staged by analogy to Musshofs suggestion from 1975. Primary extranodal Hodgkin's is extremely rare.  

Nodal lymphomas

Stage I: Disease in the lymph node region (spleen, thymus, and Waldeyer's ring are considered as nodal sites).

Stage II: Disease in two or more lymph node regions on the same side as the diaphragm, or one or more lymph node regions on the same side of the diaphragm with infiltration of adjacent extralymphatic organ/tissue (IIE).

Stage II1: Involvement of two neighboring regions which would naturally be included into one radiation field (this term is not in use for HL). 

 

Stage III: Disease in the lymph node region on both sides of the diaphragm or one or more lymph node regions on both sides of the diaphragm with infiltration of extralymphatic organ/tissue (IIIE).

Stage IV: Diffuse or disseminated disease in one or more extralymphatic organs/tissues with or without involvement of lymph nodes.  

 

Primary extranodal lymphoma

Pe I: Primary involvment of extranodal organ/tissue.

Pe IIE: Primary involvement of extranodal organ/tissue with invasion of other organ/tissue. 

Pe II1: Primary involvement of extranodal organ with spreading to regional lymph nodes.

Pe II1E: Primary involvement of extranodal organ with spreading to regional lymph nodes and with invasion of other organ/tissue. 

Pe II2: Primary involvement of extranodal organ with spreading to lymph nodes outside regional lymph nodes, but on the same side of the diaphragm. 

Pe II2E: Primary involvement of extranodal organ with spreading to lymph nodes outside regional lymph nodes, but on the same side as the diaphragm, as well as invasion of other extranodal organ/tissue. Primary extranodal lesions or lymphoma extension to extranodal lesions are seen more often with aggressive lymphomas (about 40%) than for indolent lymphomas (around 25%).

 

 

 

 

Information additional to Ann Arbor

Extranodal growth

Marked with suffix E. Localized involvement of tissue/organ in close proximity to involved lymph nodes such that direct growth - per continuitatem - is assumed.

Bulky disease

Marked with suffix X (diameter ³ 10 cm).

None/with general symptoms

Marked with suffix A/B.

Spreading-organ
(Stage IV)

Marked with first letter of the organ, for example: Stage IV L/H where L stands for lungs and H stands for liver.

Size of lymph nodes

Lymph nodes in thorax/abdomen/pelvis with smallest transversal diameter at minimum > 1.0 cm are considered pathologically enlarged. 

 

Distribution of Hodgkin's lymphoma stages
Stage Hodgkin's lymphoma
I 21%
II 33%
III 23%
IV 23%

(Norwegian national guidelines for diagnostics and treatment of malignant lymphoma, 2010) 

Subgroup A or B

Patients are classified in subgroups A or B. Subgroup A indicates the patient has not had general symptoms. Subgroup B (B symptoms) have one or more of the following general symptoms: 

  • Unexplained weight loss of more than 10% of body weight within the last 6 months.
  • Unexplained persistent or recurrent fever with temperature over 38°C in the last month
  • Repeated serious night sweats in the last month. These patients must often change bed linens multiple times per night. 

General health status

General health status is an important prognostic factor in line with staging and B symptoms.

WHO performance status is used and is staged accordingly:

  • 0: Able to carry out all normal activities without restrictions.
  • 1: Restricted in physicaly strenuous activity but ambulatory and able to carry out work of a light or sedentary nature. 
  • 2: Ambulatory and capable of all self care but unable to carry out any work activities. Up and about more than 50% of waking hours.
  • 3: Capable of only limited self-care, confined to bed or chair 50% or more of waking hours. 
  • 4: Completely disabled. Cannot carry out any self-care.

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