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Hodgkin Lymphomas

Abstract

At the present time it is not considered practical to propose a TNM classification for Hodgkin lymphoma.

Following the development of the Ann Arbor classification for Hodgkin lymphoma in 1971, the significance of two important observations with major impact on staging has been appreciated. First, extralymphatic disease, if localized and related to adjacent lymph node disease, does not adversely affect the survival of patients. Second, laparotomy with splenectomy has been introduced as a method of obtaining more information on the extent of the disease within the abdomen.

A stage classification based on information from histopathological examination of the spleen and lymph nodes obtained at laparotomy cannot be compared with another without such exploration. Therefore, two systems of classification are presented, a clinical (cS) and a pathological (pS).

1. Introductory Notes

At the present time it is not considered practical to propose a TNM classification for Hodgkin lymphoma.

Following the development of the Ann Arbor classification for Hodgkin lymphoma in 1971, the significance of two important observations with major impact on staging has been appreciated. First, extralymphatic disease, if localized and related to adjacent lymph node disease, does not adversely affect the survival of patients. Second, laparotomy with splenectomy has been introduced as a method of obtaining more information on the extent of the disease within the abdomen.

A stage classification based on information from histopathological examination of the spleen and lymph nodes obtained at laparotomy cannot be compared with another without such exploration. Therefore, two systems of classification are presented, a clinical (cS) and a pathological (pS).

2. Clinical Staging (cS)

Although recognized as incomplete, this is easily performed and should be reproducible from one centre to another. lt is determined by history, clinical examination, imaging, blood analysis, and the initial biopsy report. Bone marrow biopsy must be taken from a clinically or radiologically non-involved area of bone.

3. Liver Involvement

Clinical evidence of liver involvement must include either enlargement of the liver and at least an abnormal serum alkaline phosphatase level and two different liver function test abnormalities, or an abnormal liver demonstrated by imaging and one abnormal liver function test.

4. Spleen Involvement

Clinical evidence of spleen involvement is accepted if there is palpable enlargement of the spleen confirmed by imaging.

5. Lymphatic and Extralymphatic Disease

The lymphatic structures are as follows:
  • Lymph nodes
  • Waldeyer ring
  • Spleen
  • Appendix
  • Thymus
  • Peyer patches

The lymph nodes are grouped into regions and one or more (2, 3, etc.) may be involved. The spleen is designated S and extralymphatic organs or sites E.

6. Lung Involvement

Lung involvement limited to one lobe, or perihilar extension associated with ipsilateral lymphadenopathy, or unilateral pleural effusion with or without lung involvement but with hilar lymphadenopathy is considered as localized extralymphatic disease.

7. Liver Involvement

Liver involvement is always considered as diffuse extralymphatic disease.

8. Pathological Staging (pS)

This takes into account additional data and has a higher degree of precision. lt should be applied whenever possible. A - (minus) or + (plus) sign should be added to the various symbols for the examined tissues, depending on the results of histopathological examination.

9. Histopathological Information

This is classified by symbols indicating the tissue sampled. The following notation is common to the distant metastases (or M1 categories) of all regions classified by the TNM system. However, in order to conform with the Ann Arbor classification, the initial letters used in that system are also given.

Pulmonary
PUL or L
Bone marrow
MAR or M
Osseous
OSS or O
Pleura
PLE or P
Hepatic
HEP or H
Peritoneum
PER
Brain
BRA
Adrenals
ADR
Lymph nodes
LYM or N
Skin
SKI or D
Others
OTH


10. Clinical Stages (cS)

10.1. Stage I Involvement of a single lymph node region (I), or localized involvement of a single extralymphatic organ or site (IE)

10.2. Stage II Involvement of two or more lymph node regions on the same side of the diaphragm (II), or localized involvement of a single 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 (e.g.II3)

10.3. Stage III 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), or by involvement of the spleen (IIIS), or both (IIIE+S)

10.4. Stage IV Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement with distant (non-regional) nodal involvement

Note: The site of Stage IV disease is identified further by specifying sites according to the notations listed above.

11. A and B Classification (Symptoms)

Each stage should be divided into A and B according to the absence or presence of defined general symptoms. These are:

1. Unexplained weight loss of more than 10% of the usual body weight in the 6 months prior to first attendance

2. Unexplained fever with temperature above 38°C

3. Night sweats

Note: Pruritus alone does not qualify for B classification nor does a short, febrile illness associated with a known infection.

12. Pathological Stages (pS)

The definitions of the four stages follow the same criteria as the clinical stages but with the additional information obtained following laparotomy. Splenectomy, liver biopsy, lymph node biopsy, and marrow biopsy are mandatory for the establishment of pathological stages. The results of these biopsies are recorded as indicated above (see Pathological Staging (pS) and Clinical Stages (cS)).

13. pTNM Pathological Classification

14. Summary

Stage
Hodgkin Lymphoma
Substage
Stage I
Single node region


Localized single extralymphatic organ/site
IE
Stage II
Two or more node regions, same side of diaphragm


Localized single extralymphatic organ/ site with its regional nodes, ± other node regions same side of diaphragm
IIE
Stage III
Node regions both sides of diaphragm ± Localized single extralymphatic

organ/site
IIIE
Spleen
IIIS
Both
IIIE+S
Stage IV
Diffuse or multifocal involvement of extralymphatic organ(s) ± regional nodes; isolated extralymphatic organ and non-regional nodes

All stages divided
Without weight loss/fever/sweats
A
With weight loss/fever/sweats
B