HODGKIN LYMPHOMA

Introductory Notes

The current staging classification for Hodgkin Lymphoma is a modification of the Ann Arbor classification first adopted in 1971. Over the past 50 years the practice has changed, making the previously used staging laparotomy and the resulting pathological staging classification obsolete. The consensus conference that took place in 2012 in Lugano suggested an even more simplified system, putting together Stages I and II as Limited Stage and Stages III and IV as Advanced Stage lymphoma. The Lugano Classification, a modification of the Ann Arbor classification, has been published and accepted by the UICC.¹

Clinical Staging (cS)

This 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.

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.

Spleen Involvement

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

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.

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.

Liver Involvement

Liver involvement is always considered as diffuse extralymphatic disease.

Clinical Stages (cS)

Limited Stage

Stage I

Involvement of a single lymph node region (I) (Figs. 550, 551, 552, 553) or localized involvement of a single extralymphatic organ or site (IE) (Fig. 554).

Schematic illustration of Involvement of a single lymph node region. — **Fig. 550**

Schematic illustration of Involvement of a single lymph node region tonsil. — **Fig. 551**

Schematic illustration of Involvement of a single lymph node region spleen. — **Fig. 552**

Schematic illustration of Involvement of a single lymph node region thymus. — **Fig. 553**

Schematic illustration of localized involvement of a single extralymphatic organ Lung. — **Fig. 554**

Stage II

Involvement of two or more lymph node regions on the same side of the diaphragm (II) (Fig. 555), 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) (Fig. 556).

Schematic illustration of Involvement of two or more lymph node regions on the same side of the diaphragm. — **Fig. 555**

Schematic illustration of 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. — **Fig. 556**

Bulky Stage II

Stage II disease with a single nodal mass greater than 10 cm in maximum dimension or greater than a third of the thoracic diameter as assessed on CT (Figs. 557, 558, 559).

Schematic illustration of Stage II disease with a single nodal mass greater than 10 cm in maximum dimension or greater than a third of the thoracic diameter as assessed on CT. — **Fig. 557**

Photo depicts Stage II disease with a single nodal mass greater than 10 cm in maximum dimension or greater than a third of the thoracic diameter as assessed on CT. — **Fig. 558**

Advanced Stage

Stage III

Involvement of lymph node regions on both sides of the diaphragm (III) (Fig. 560), which may also be accompanied by localized involvement of an associated extralymphatic organ or site (IIIE) (Fig. 561), or by involvement of the spleen (IlIS), or both (IIIE + S) (Fig. 562).

Schematic illustration of Involvement of lymph node regions on both sides of the diaphragm (III). — **Fig. 560**

Schematic illustration of 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. — **Fig. 561**

Schematic illustration of Involvement of lymph node regions on both sides of the diaphragm (III), which may also be accompanied by involvement of the spleen (IlIS), or both (IIIE + S). — **Fig. 562**

Stage IV

Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated lymph node involvement (Figs. 563, 564); or non‐contiguous extralymphatic organ involvement with involvement of lymph node regions on the same or both sides of the diaphragm (Fig. 565).

Note

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

Schematic illustration of Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated lymph node involvement. — **Fig. 563**

Schematic illustration of Disseminated (multifocal) involvement of one or more extralymphatic organs, with or without associated non‐contiguous extralymphatic organ involvement with involvement of lymph node regions on the same or both sides of the diaphragm. — **Fig. 565**

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:

Unexplained weight loss of more than 10% of the usual body weight in the 6 months prior to first attendance
Unexplained fever with temperature above 38 °C
Night sweats

Note

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