Non-Hodgkin lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Non-Hodgkin lymphoma may be classified into subtypes according to updated WHO classification and rate of growth.

Classification

A. Updated WHO classification

  • Non-Hodgkin lymphoma may be classified according to updated WHO classification into 2 groups:[1]
  • B-cell neoplasms
  • T-cell and putative NK-cell neoplasms
Updated WHO classification of Non-Hodgkin lymphoma
B-cell neoplasms T-cell and putative NK-cell neoplasms
Precursor B-cell neoplasm Precursor T-cell neoplasm
Precursor B-acute lymphoblastic leukemia / lymphoblastic lymphoma (LBL) Precursor T-acute lymphoblastic leukemia / lymphoblastic lymphoma (LBL)
Mature B-cell neoplasms Peripheral T-cell and NK-cell neoplasms
Chronic lymphocytic leukemia / small lymphocytic lymphoma T-cell CLL / prolymphocytic leukemia
Monoclonal B-cell lymphocytosis
B-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia
Splenic marginal zone lymphoma (± villous lymphocytes)

Splenic B-cell lymphoma/leukemia, unclassifiable

1. Splenic diffuse re pulp small B-cell lymphoma

2. Hairy cell leukemia-variant

Extranodal T-/NK-cell lymphoma, nasal type
Hairy cell leukemia Enteropathy-type intestinal T-cell lymphoma
Lymphoplasmacytic lymphoma

Waldenström's macroglubulinemia

Mycosis fungoides / Sézary syndrome
Monoclonal gammopathy of undetermined significance (MGUS),
  1. IgM*

2. IgG/A

µ heavy-chain disease

ɣ heavy-chain disease

α heavy-chain disease

Plasma cell myeloma (multiple myloma)
Solitary plasmacytoma of bone
Extraosseous plasmacytoma
Monoclonal immunoglobulin deposition diseases
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
Nodal marginal zone B-cell lymphoma (± monocytoid B-cells)

pediatric Nodal marginal zone B-cell lymphoma

Angioimmunoblastic T-cell lymphoma
Follicular lymphoma

In situ follicular neoplasia

Duodenal-type follicular lymphoma

Pediatric-type follicular lymphoma

Hepatosplenic gamma / delta T-cell lymphoma
Large B-cell lymphoma with IRF4 rearrangement
Primary cutaneous follicle center lymphoma Subcutaneous panniculitis-like T-cell lymphoma
Mantle cell lymphoma

In situ mantle cell neoplasia

Peripheral T-cell lymphoma, not otherwise characterized
Diffuse large B-cell lymphoma (DLBCL), NOS

Germinal center B-cell type

Activated B-cell type

Primary DLBCL of the central nervous system (CNS)

Primary cutaneous DLBCL, leg type

DLBCL associated with chronic inflammation

HHV81 DLBCL, NOS

Anaplastic large cell lymphoma, primary systemic type
T-cell/histiocyte-rich large B-cell lymphoma
EBV1 DLBCL, NOS

EBV1 mucocutaneous ulcer

Adult T-cell lymphoma / leukemia (human T-lymphotrophic virus [HTLV] 1+)
Lymphomatoid granulomatosis
Primary mediastinal (thymic) large B-cell lymphoma Aggressive NK-cell leukemia
Intravascular large B-cell lymphoma
ALK1 large B-cell lymphoma
Plasmablastic lymphoma
Primary effusion lymphoma
Burkitt lymphoma

Burkitt-like lymphoma with 11q aberration

Anaplastic large cell lymphoma, primary cutaneous type
High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements

High-grade B-cell lymphoma, NOS*

B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma

B. Classification based on rate of growth

  • Non-Hodgkin lymphoma may be classified based on rate of growth into 2 groups:[2]
  • Low-grade or Indolent lymphoma
  • High-grade or Aggressive lymphoma
Non-Hodgkin lymphoma classification
Grade Description
Low-grade or Indolent lymphoma
  • Tend to grow very slowly
  • Tend to be widespread by the time they are diagnosed
  • Often involving the bone marrow and spleen
  • Often treated only when symptoms appear
  • Can shrink or seem to disappear with treatment, but they tend to come back
  • Can change into more aggressive lymphomas
  • Have a fairly good prognosis
High-grade or Aggressive lymphoma
  • Grow quickly and tend to spread to lymph nodes or other organs throughout the body
  • Cause symptoms and need treatment right away
  • Can frequently be successfully treated with intensive chemotherapy treatment
Non-Hodgkin's Lymphoma

References

  1. National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/publications/pdq
  2. "Canadian Cancer Society Grades of non-Hodgkin lymphoma".

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