Hodgkin Lymphoma Staging

Updated: Dec 28, 2020
Author: Francisco J Hernandez-Ilizaliturri, MD; Chief Editor: Matthew C Foster, MD 

Classification and Staging Systems for Hodgkin Lymphoma

The World Health Organization (WHO)/Revised European-American Lymphoma (REAL) classification[1] and the Cotswolds modified Ann Arbor staging system for Hodgkin lymphoma (HL) are provided below[2] , as well as the risk stratification for HL.

WHO/REAL classification

Nodular lymphocyte-predominant HL:

Classic HL is grouped into the following 4 types:

  • Nodular sclerosis classic HL
  • Lymphocyte-rich classic HL
  • Mixed cellularity classic HL
  • Lymphocyte-depleted classic HL

For therapeutic purposes, nodular lymphocyte-predominant HL is managed in the same way as indolent non-Hodgkin lymphoma (see Follicular Lymphoma [non-Hodgkin Lymphoma]).[1]

Table 1. Cotswold Modification of Ann Arbor Staging System (Open Table in a new window)

Stage

Area of Involvement

I

Single lymph node group

II

Multiple lymph node groups on same side of diaphragm

III

Multiple lymph node groups on both sides of diaphragm

IV

Multiple extranodal sites or lymph nodes and extranodal disease

X

Bulk > 10 cm

E

Extranodal extension or single, isolated site of extranodal disease

A/B

B symptoms: weight loss > 10%, fever, drenching night sweats

The Cotswolds modification maintains the original 4-stage clinical and pathologic staging framework of the Ann Arbor staging system but also adds information regarding the prognostic significance of bulky disease (denoted by an X designation) and regions of lymph node involvement (denoted by an E designation).[2]

The A and B designations denote the absence or presence of symptoms, respectively; the presence of symptoms correlates with treatment response. The importance of imaging modalities, such as computed tomography (CT) scanning, is also underscored.[2]

Risk stratification in HL

Based on the clinical scenario, staging and degree of end-organ damage in patients with HL can be categorized into the following 3 groups (this classification has impact in treatment selection and must be performed carefully in every patient with HL):

  • Early-stage favorable
  • Early-stage unfavorable (bulky and nonbulky)
  • Advanced-stage 

The following table shows examples of unfavorable risk factors for stages I and II. Data are included from 3 organizations, including the German Study Hodgkin Lymphoma Study Group (GSHG), European Organization for Research and Treatment of Cancer (EORTC), and the National Cancer Institute of Canada (NCIC).

Table 2. Unfavorable Risk Factors for Stages I and II Hodgkin Lymphoma (Open Table in a new window)

Risk Factor

GSHG

EORTC

NCIC

Age

 

≥ 50y

≥ 40y

Histology

 

MC or LD

 

ESR or B symptoms

> 50 if A or > 30 if B

> 50 if A or > 30 if B

> 50 or any B symptoms

Mediastinal mass*

MMR > 0.33

MMR > 0.35

MMR > 0.33 or > 10 cm

Number of nodal sites

> 2

> 3

> 3

Extranodal lesions

any

 

 

* Mediastinal mass is measured on chest x-ray by the mediastinal mass ratio (MMR), which is defined by the following: maximum width of mass/maximum intrathoracic diameter.

ESR = erythrocyte sedimentation rate; LD = lymphocyte depletion; MC = mixed cellularity.

Based on the criteria described above, patients are then classified as follows:

  • Early-stage favorable HL (includes patients with stage I or II HL and no risk factors by GSHG/EORTC or NCIC)
  • Early-stage unfavorable HL (includes patients with stage I and II HL and one or more risk factors) with non-bulky disease
  • Early-stage unfavorable HL with non-bulky disease
  • Advanced-stage HL (includes patients with stages IIB, III, and IV)

Patients with advanced disease are further risk stratified using the International Prognostic Score (IPS), which includes the following risk factors (for each present factor, the patient receives 1 point)[3] :

  • Albumin < 4 g/dL
  • Hemoglobin < 10.5 g/dL
  • Male
  • Age ≥ 45y
  • Stage IV disease
  • Leukocytosis: white cell count (WBC) > 15,000/μL
  • Lymphopenia: lymphocyte count < 8% of WBC count and/or absolute lymphocyte count < 600 cells/μL

Based on the IPS score, patients with advanced disease can be categorized as follows[3] :

  • Good risk (IPS 0-1)
  • Fair risk (IPS 2-3)
  • Poor risk (IPS 4-7)