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Staging of lymphoma

Hodgkin's lymphoma

The Ann Arbor classification (1971) is used with the recent Cotswold revision.  

Stage I: Involvement of lymph node region (spleen, thymus, and Waldeyer ring are considered nodal involvement).

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm. 

 

 

Stage III: Involvement of lymph node regions on both sides of the diaphragm.

Stage IV: Diffuse or disseminated involvement in one or more extralymphatic organ/tissue with or without involvement of lymph nodes. 

 

Additions to Ann Arbor classification

Extranodal growth

Denoted with suffix E. Localized involvement of tissue/organ in close proximity to involved lymph nodes such that direct tissue invasion is assumed.

Bulky

Denoted by X (diameter ≥ 10 cm).

None/with general symptoms

Denoted by A/B.

Spreading-organ (Stage IV)

Denoted with first letter of organ, for example: Stage IV L/H where L stands for lungs and H stands for hepatic.

Lymph node size

Lymph nodes in thorax/abdomen/pelvis with diameter > 1.0 cm as pathologically enlarged

Subgroup A or B

Patients are classified in subgroup A or B based on symptoms. Subgroup A indicates the absence of defined general symptoms. 

Subgroup B (B-symptoms) has one or more of the following symptoms:

  • Unexplained weight loss of more than 10% of body weight in the 6 last months
  • Unexplained persisting or recurring fever with temperature over 38°C the last 6 months.
  • Repeated night sweats the last month - these patients have drenching sweats and must often change bed sheets or nightshirts multiple times during the night.

Therapy groups

In Norway, it is standard to apply treatment from protocols of the Deutsche Arbeitsgemeinschaft für Leukämieforschung und -behandlung im Kindesalter (the DAL-HD-protocols) and later Gesellschaft für Pädiatrische Onkologie und Hämatologie (the GPOH-HD-95 protocols). Three therapy groups are defined based on stages. 

Therapy groups according to GPOH-HD-95

Therapy groups

Stage

TG-1 I A/B and II A
TG-2 IEA/B, IIEA, II B or III A
TG-3 IIEB, IIIEA/B, III B or IV A/B

Non-Hodgkin lymphoma

For staging of childhood non-Hodgkins lymphoma, the St. Jude classification is used. Treatment intensity and length varies based on the stage of the disease as well as the prognosis.  

St. Jude Staging System
Stage I

A single nodal or extranodal tumor manifestation without local spreading, except for mediastinal, abdominal, or epidural localizations.

Stage II Multiple nodal or extranodal manifestations on the same side of the diaphragm, with or without local spreading, except for mediastinal, epidural or extensive non-resectable abdominal localizations.
Stage III
  • Localizations on both sides of the diaphragm
  • All thoracic manifestations (mediastinum, thymus, pleura)
  • All widespread non-resectable abdominal localizations
  • Epidural invasion
  • Multifocal skeletal invasion
Stage IV Bone marrow and/or CNS invasion

Risk groups

B-cell lymphoma and large cell anaplastic lymphoma are also separated into risk groups: The risk group determines the choice of treatment.   

B cell  lymphoma (B-NHL BMF 04)

Risk group 1

Macroscopically completely removed tumor

Risk group 2

Resection of tumor is incomplete. This may be stage I or II disease, as well as stage III disease with an LD < 500 U/l

Risk group 3

Resection of the tumor manifestation is incomplete. This is a stage III disease with LD from 500 U/l to 1000 U/l, as well as stage IV/B-ALL, LD < 1000 U/l and CNS negative.

Risk group 4

The resection of the tumor is incomplete. This is a stage III disease with LD ≥ 1000 U/l, as well as stage IV/B-ALL, LD ≥ 1000 U/l and or CNS positive

Large cell anaplastic lymphoma (ALCL 99)
Low risk group Stage I disease has been completely resected.
Standard risk group
  • No skin infiltration
  • No mediastinal tumor
  • No involvement of liver, spleen, or lungs
High risk group

Includes patients with the following:

  • Skin lesions proven by biopsy (with exception of skin lesions lying over an infiltrated lymph node or isolated skin disease with < 5 lesions)
  • Mediastinal tumor
  • Liver (enlarged ≥ 5 cm and/or nodes in liver), spleen (enlarged spleen and/or nodes in spleen) or lung infiltration
  • Less than 5 isolated skin lesions are not considered high risk factors. 
  • Large cell anaplastic lymphoma limited to the skin is uncommon in children and may be a special variant of the disease. The treatment should be decided individually. 

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