Non-Hodgkin lymphoma classification

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

## Overview

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

## Classification

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### A. Updated WHO classification (2016)

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* Non-Hodgkin lymphoma may be classified according to updated WHO classification into 2 groups:[1][2]

* 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 prolymphocytic leukemia
Monoclonal B-cell lymphocytosis | T-cell granular lymphocytic leukemia
B-cell prolymphocytic leukemia | Chronic lymphoproliferative disorder of NK cells
Splenic marginal zone lymphoma (± villous lymphocytes)

Splenic B-cell lymphoma/leukemia, unclassifiable

1\. Splenic diffuse red pulp small B-cell lymphoma

2\. Hairy cell leukemia-variant

| Aggressive NK-cell leukemia
Hairy cell leukemia | Systemic EBV positive T-cell lymphoma of childhood
Lymphoplasmacytic lymphoma

Waldenström's macroglubulinemia

| Hydroa vacciniforme like lymphoproliferative disorder
Monoclonal gammopathy of undetermined significance (MGUS),

* IgM
* IgG/A

| Adult T-cell leukemia/lymphoma
Heavy chain disease

* µ heavy-chain disease
* ɣ heavy-chain disease
* α heavy-chain disease

| Extranodal T/NK-cell lymphoma, nasal type
Plasma cell myeloma (multiple myloma)  | Enteropathy associated intestinal T-cell lymphoma
Solitary plasmacytoma of bone  | Monomorphic epitheliotropic intestinal T-cell lymphoma
Extraosseous plasmacytoma  | Indolent T-cell lymphoproliferative disorder of the GI tract
Monoclonal immunoglobulin deposition diseases  | Hepatosplenic T-cell lymphoma
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)  | Subcutaneous panniculitis-like T-cell lymphoma
Nodal marginal zone B-cell lymphoma (± monocytoid B-cells)

pediatric

Nodal marginal zone B-cell lymphoma

| Mycosis fungoides
Follicular lymphoma

* In situ follicular neoplasia
* Duodenal-type follicular lymphoma
* Pediatric-type follicular lymphoma

| Sézary syndrome
Large B-cell lymphoma with IRF4 rearrangement  | Primary cutaneous CD30 T-cell lymphoproliferative disorders

* Lymphomatoid papulosis
* Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous follicle center lymphoma | Primary cutaneous gamma delta T-cell lymphoma

Primary cutaneous CD8 aggressive epidermotropic cytotoxic T-cell lymphoma

Primary cutaneous acral CD8 T-cell lymphoma

Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder

Mantle cell lymphoma

In situ mantle cell neoplasia

| Peripheral T-cell lymphoma, NOS *

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

| Angioimmunoblastic T-cell lymphoma
T-cell/histiocyte-rich large B-cell lymphoma  | Follicular T-cell lymphoma
EBV1 DLBCL, NOS

EBV1 mucocutaneous ulcer

| Nodal peripheral T-cell lymphoma with TFH phenotype
Lymphomatoid granulomatosis | Anaplastic large cell lymphoma

* ALK positive
* ALK negitive

Primary mediastinal (thymic) large B-cell lymphoma | Breast implant associated anaplastic large-cell lymphoma
Intravascular large B-cell lymphoma |
ALK1 large B-cell lymphoma  |
Plasmablastic lymphoma  |
Primary effusion lymphoma |
Burkitt lymphoma

Burkitt-like lymphoma with 11q aberration

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

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* Non-Hodgkin lymphoma may be classified based on rate of growth into 2 groups:[3]

* 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

## References

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1. ↑ Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
2. ↑ National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/publications/pdq
3. ↑ "Canadian Cancer Society Grades of non-Hodgkin lymphoma".

Template:Hematology

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Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)

CFU-GM/
and other granulocytes|

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MEP|

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CFU-Mast|

Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)

Multiple/unknown|

AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) * MP (Myelofibrosis) * Acute biphenotypic leukaemia

See also hematology, lymphoid malignancy

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