Lymphoma Classification:
Types and Locations (updated)

NHL Categories

The older Rappaport, Working Formulation, and REAL categories are described in a separate section for reference.  This might be helpful if a patient's records state some of the older lymphoma classifications.

Unlike the IWF and REAL classifications, the WHO committee felt that grouping lymphomas according to behavior (indolent, intermediate, aggressive) was neither necessary nor desirable. The committee recognized that specific disease entities could be defined by a combination of morphology, immunology, genetic features, and clinical features. Each entity had distinct clinical behavior and outcome predictable by applicable prognostic factors (e.g.; the international Prognostic Index) and related to the type of initial therapy administered. The committee concluded that each lymphoma type needed to be treated as distinct entities. Therefore, rather than depending on clinical grouping (i.e.; low grade/indolent, etc.), the committee emphasized that clinical decisions should be based on the specific lymphoid neoplasm (type of lymphoma).

B-Cell Neoplasms

I. Precursor B-cell neoplasm:
    a. Precursor B-lymphoblastic leukemia/lymphoma

II. Mature (peripheral) B-cell neoplasms

1. B-cell chronic lymphocytic leukemia / 
      small lymphocytic lymphoma
2. B-cell prolymphocytic leukemia
3. Lymphoplasmacytic lymphoma
4. Splenic marginal zone B-cell lymphoma (+/- villous lymphocytes)
5. Nodal marginal zone lymphoma (+/- monocytoid B-cells)
6. Extranodal marginal zone B-cell lymphoma of
    mucosa-associated lymphoid tissue (MALT) type
7. Hairy cell leuekmia
8. Plasma cell myeloma/plasmacytoma
9. Follicular lymphoma, follicle center
10. Mantle cell lymphoma
11. Diffuse large cell B-cell lymphoma
    • Mediastinal large B-cell lymphoma
    • Intravascular large B-cell lymphoma
    • Primary effusion lymphoma
12. Burkitt's lymphoma/Burkitt's cell leukemia

B-cell proliferations of uncertain malignant potential 

• Lymphomatoid granulomatosis 
• Post-transplant lymphoproliferative disorder

T-Cell and Natural Killer Cell Neoplasms

I. Precursor T cell neoplasm:
    a.Precursor T-lymphoblastic lymphoma/leukemia
         b. Blastic NK lymphoma  

II. Mature (peripheral) T cell and NK-cell neoplasms

1. T cell prolymphocytic leukemia
2. T-cell granular lymphocytic leukemia
3. Aggressive NK Cell leukemia
4. Adult T cell lymphoma/leukemia (HTLV1+)
5. Extranodal NK/T-cell lymphoma, nasal type  
6. Enteropathy-type T-cell lymphoma
7. Hepatosplenic gamma-delta T-cell lymphoma
8. Subcutaneous panniculitis-like T-cell lymphoma
9. Mycosis fungoides/Sézary's syndrome
10. Primary Cutaneous Anaplastic large cell lymphoma T/null cell
11. Peripheral T cell lymphoma, unspecified
12. Angioimmunoblastic T cell lymphoma
13. Primary Systemic Anaplastic large cell lymphoma, T/null cell

T-cell proliferation of uncertain malignant potential 

•  Lymphomatoid papulosis

Hodgkin's Lymphoma (Hodgkin's Disease) (B Cell Origin)

1. Nodular lymphocyte predominance Hodgkin's lymphoma
2. Classical Hodgkin's lymphoma
   • Nodular sclerosis Hodgkin's lymphoma
   • Lymphocyte-rich classical Hodgkin's lymphoma
   • Mixed cellularity Hodgkin's lymphoma
   • Lymphocyte depletion Hodgkin's lymphoma

Lymphomas by Location

• Central Nervous System (CNS) Lymphoma
• Lymphoma of Bone
• Eyelid Lymphoma (new)
• Digestive Tract Lymphoma
• Ocular (Eye) Lymphoma (new)

Multimedia Resources (new)

• What are the Different Types of Non-Hodgkin's Lymphoma? - Video with Dr. John Hainsworth, MD
• Lymphoma Classification: Making Sense of a Complex Disease - article by Amy Chadburn, MD

References

• The National Cancer Institute NHL, SEER List with ICD-O Codes  
• Who's W.H.O. on Lymphomas by Patrick Treseler, MD PhD (new)
• 1998 paper: New Approach to Classifying Non-Hodgkin's Lymphomas: Clinical Features of the Major Histologic Subtypes by Armitage and Weisenburger for the NHL Classification Project
• The Changing Classification of Non-Hodgkin's Lymphomas by JO
  Armitage in the Cancer Journal 
• A good but technical overview of all classification types comes from
  Dr. David Wiessmann  

• Lymphoma Information Network