Diffuse Large B-Cell Lymphoma (Non-Hodgkin Lymphoma) Staging 

Updated: Apr 15, 2016
  • Author: Francisco J Hernandez-Ilizaliturri, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Staging of Diffuse Large B-Cell Lymphoma

Systems for staging diffuse large B-cell lymphoma (DLBCL) are provided below, including the World Health Organization (WHO)/Revised European American Classification of Lymphoid Neoplasms (REAL) system [1, 2] and the Cotswolds modification of the Ann Arbor system. Risk stratification with the International Prognostic Index (IPI) score is also provided. [3]

WHO/REAL classification

DLBCL, not otherwise specified (NOS):

  • T-cell/histiocyte rich large B-cell lymphoma
  • Primary DLBCL of the central nervous system (CNS)
  • Primary cutaneous DLBCL, leg type
  • Epstein-Barr virus (EBV)-positive DLBCL of the elderly
  • Germinal center B-cell (GCB)-DLBCL*
  • Activated B-cell (ABC)-DLBCL­­*

DLBCL associated with chronic inflammation:

  • Primary mediastinal lymphoma­­
  • Intravascular large B-cell lymphoma
  • Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma

* These 2 types of lymphoma are not yet recognized by the WHO 2008 classification. However, they are distinct subtypes of DLBCL on the basis of the cell of origin and represent distinct variants of DLBCL, with different biologies and clinical prognoses. [2]

Table. Cotswolds 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
II 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

Risk stratification (IPI score)

Factors (1 point for each factor present):

  • Age ≥ 60y
  • Eastern Cooperative Oncology Group (ECOG) performance status ≥ 2
  • Elevated lactate dehydrogenase (LDH)
  • Ann Arbor stage III or IV
  • ≥ 2 extranodal sites of disease

Risk category (factors):

  • Low (0 or 1)
  • Low-intermediate (2)
  • High-intermediate (3)
  • High (4 or 5)