Diffuse large B cell lymphoma classification: Difference between revisions

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*[[prognosis]] is variable
*[[prognosis]] is variable


===Other Large B Cell Lymphoma (DLBCL) type===     
===Other Large B Cell Lymphoma (DLBCL) type===
===='''T-cell/histiocyte-rich large B cell lymphoma (rare)'''====     


*'''T-cell/histiocyte-rich large B cell lymphoma (rare)'''
*Few large [[B cell|B cells]] embedded in a background of [[T cell|T cells]] and histiocytes; distinguish from [[nodular]] [[lymphocyte]]-predominant [[Hodgkin's lymphoma|Hodgkin lymphoma]]
*Few large [[B cell|B cells]] embedded in a background of [[T cell|T cells]] and histiocytes; distinguish from [[nodular]] [[lymphocyte]]-predominant [[Hodgkin's lymphoma|Hodgkin lymphoma]]
*Commonly found in middle-aged men, advanced stage with extranodal involvement ([[liver]], [[spleen]], [[bone marrow]]); poor prognosis
*Commonly found in middle-aged men, advanced stage with extranodal involvement ([[liver]], [[spleen]], [[bone marrow]]); poor prognosis
*
*
*'''Primary diffuse large B cell lymphoma of the central nervous system (rare)'''
 
==== '''Primary diffuse large B cell lymphoma of the central nervous system (rare)''' ====
 
*Typically ABC subtype; frequent loss of [[Human leukocyte antigen|HLA]] class I/II; frequent mutation of ''MYD88''
*Typically ABC subtype; frequent loss of [[Human leukocyte antigen|HLA]] class I/II; frequent mutation of ''MYD88''
*Exclusively in [[CNS]] or [[Intraocular lymphoma|intraocular]] region, rare systemic involvement; poor [[prognosis]]; specialized treatment with [[CNS]]-penetrating agents, with or without [[radiation]] therapy, required; targeted therapies under [[Investigational drug|investigation]]
*Exclusively in [[CNS]] or [[Intraocular lymphoma|intraocular]] region, rare systemic involvement; poor [[prognosis]]; specialized treatment with [[CNS]]-penetrating agents, with or without [[radiation]] therapy, required; targeted therapies under [[Investigational drug|investigation]]
*
*
*'''Primary Cutaneous DLBCL, Leg type (rare)'''
 
==== '''Primary Cutaneous DLBCL, Leg type (rare)''' ====
 
*Typically ABC subtype; frequent mutation of [[MYD88]]; distinguish from other [[Skin|cutaneous]] [[B-cell lymphoma]]
*Typically ABC subtype; frequent mutation of [[MYD88]]; distinguish from other [[Skin|cutaneous]] [[B-cell lymphoma]]
*Typically in elderly patients and women; presents with [[skin nodules]] in lower [[Leg|legs]]; 10–15% of cases arise in other sites; poor [[prognosis]]  
*Typically in elderly patients and women; presents with [[skin nodules]] in lower [[Leg|legs]]; 10–15% of cases arise in other sites; poor [[prognosis]]
*
*
*'''EBV-positive diffuse large B-cell lymphoma, NOS (rare)'''
 
==== '''EBV-positive diffuse large B-cell lymphoma, NOS (rare)''' ====


*Variable [[Histology|histologic]] features, including [[Hodgkin's lymphoma|Hodgkin]]-like lesions, [[Monomorphic adenoma|monomorphic]] to [[Polymorphism (biology)|polymorphic]] patterns; [[Epstein Barr virus|EBV]] detectable in [[tumor]] and frequently in serum
*Variable [[Histology|histologic]] features, including [[Hodgkin's lymphoma|Hodgkin]]-like lesions, [[Monomorphic adenoma|monomorphic]] to [[Polymorphism (biology)|polymorphic]] patterns; [[Epstein Barr virus|EBV]] detectable in [[tumor]] and frequently in serum
*Typically in patients older than 50 yr; more frequent in Asia and Latin America than elsewhere; [[Extranodal NK-T-cell lymphoma|extranodal]] involvement common; [[prognosis]] varies
*Typically in patients older than 50 yr; more frequent in Asia and Latin America than elsewhere; [[Extranodal NK-T-cell lymphoma|extranodal]] involvement common; [[prognosis]] varies


*'''EBV-positive mucocutaneous ulcer (rare)'''
==== '''EBV-positive mucocutaneous ulcer (rare)''' ====
 
*[[Polymorphism (biology)|Polymorphic]] [[infiltrate]] with frequent [[Hodgkin's lymphoma|Hodgkin]]-like cells; [[Epstein Barr virus|EBV]] is detectable in [[tumor]]
*[[Polymorphism (biology)|Polymorphic]] [[infiltrate]] with frequent [[Hodgkin's lymphoma|Hodgkin]]-like cells; [[Epstein Barr virus|EBV]] is detectable in [[tumor]]
*Presents as [[Localized Lymph Node Enlargement|localized]], [[Ulcer|ulcerated]] lesions in [[oral mucosa]], [[Intestines|intestine]], or [[skin]]; dissemination is rare; commonly occurs as iatrogenic or age-related disease in [[Immunodeficiency|immunocompromised]] patients; favorable prognosis; consider reduction of [[Immunosuppression|immunosuppressive therapy]]
*Presents as [[Localized Lymph Node Enlargement|localized]], [[Ulcer|ulcerated]] lesions in [[oral mucosa]], [[Intestines|intestine]], or [[skin]]; dissemination is rare; commonly occurs as iatrogenic or age-related disease in [[Immunodeficiency|immunocompromised]] patients; favorable prognosis; consider reduction of [[Immunosuppression|immunosuppressive therapy]]
*
*
*'''Diffuse large B-cell lymphoma associated with chronic inflammation (rare)'''
 
==== '''Diffuse large B-cell lymphoma associated with chronic inflammation (rare)''' ====
 
*Morphologically similar to [[Diffuse large B cell lymphoma|DLBCL]], NOS but strongly associated with [[Epstein Barr virus|EBV]]; also called [[Pleural empyema|pyothorax]]-associated [[lymphoma]], when associated with chronic [[pyothorax]]
*Morphologically similar to [[Diffuse large B cell lymphoma|DLBCL]], NOS but strongly associated with [[Epstein Barr virus|EBV]]; also called [[Pleural empyema|pyothorax]]-associated [[lymphoma]], when associated with chronic [[pyothorax]]
*Occurs in context of [[chronic inflammation]], involving pleural cavity or other sites such as [[bone]] and [[Joint|joints]]; [[male]] predominance; poor [[prognosis]]
*Occurs in context of [[chronic inflammation]], involving pleural cavity or other sites such as [[bone]] and [[Joint|joints]]; [[male]] predominance; poor [[prognosis]]
*
*
*'''Lymphomatoid granulomatosis (rare)'''
 
==== '''Lymphomatoid granulomatosis (rare)''' ====
 
*[[Epstein Barr virus|EBV]]-driven angiocentric and angiodestructive [[lymphoproliferation]] with reactive [[T cell|T cells]]; grade based on proportion of [[Epstein Barr virus|EBV]] positive [[B cell|B cells]] and cytologic features
*[[Epstein Barr virus|EBV]]-driven angiocentric and angiodestructive [[lymphoproliferation]] with reactive [[T cell|T cells]]; grade based on proportion of [[Epstein Barr virus|EBV]] positive [[B cell|B cells]] and cytologic features
*Commonly involves extranodal sites ([[lung]] >90%); often in context of [[immunodeficiency]]; prognosis varies; no standard therapy
*Commonly involves extranodal sites ([[lung]] >90%); often in context of [[immunodeficiency]]; prognosis varies; no standard therapy
*
*
*'''Large B-cell lymphoma with ''IRF4'' rearrangement (rare)'''
 
==== '''Large B-cell lymphoma with ''IRF4'' rearrangement (rare)''' ====
 
*Strong [[expression]] of IRF4/MUM1, usually with ''[[IRF4]]'' rearrangement; [[diffuse]]-to-[[Follicular lymphoma|follicular]] [[Morphological computation|morphologic]] features; distinguish from [[Pediatrics|pediatric]]-type [[follicular lymphoma]]
*Strong [[expression]] of IRF4/MUM1, usually with ''[[IRF4]]'' rearrangement; [[diffuse]]-to-[[Follicular lymphoma|follicular]] [[Morphological computation|morphologic]] features; distinguish from [[Pediatrics|pediatric]]-type [[follicular lymphoma]]
*Commonly in [[Child|children]] and [[Young adult|young adults]]; typically involves Waldeyer’s ring or [[cervical lymph nodes]]; favorable prognosis
*Commonly in [[Child|children]] and [[Young adult|young adults]]; typically involves Waldeyer’s ring or [[cervical lymph nodes]]; favorable prognosis
*
*
*'''Primary mediastinal (thymic) large B-cell lymphoma (around 6% of large B-cell cases)'''
 
==== '''Primary mediastinal (thymic) large B-cell lymphoma (around 6% of large B-cell cases)''' ====
 
*Putative [[Thymus|thymic]] [[B cell|B-cell]] origin; medium-to-large [[B cell|B cells]], frequently with [[sclerosis]]; distinctive [[phenotype]] ([[CD30]], [[CD23]], [[PDL1]], [[PDL2]]) and unique GEP signature; frequent 9p21 [[amplification]], [[genomic]] alterations of ''[[CIITA]]''
*Putative [[Thymus|thymic]] [[B cell|B-cell]] origin; medium-to-large [[B cell|B cells]], frequently with [[sclerosis]]; distinctive [[phenotype]] ([[CD30]], [[CD23]], [[PDL1]], [[PDL2]]) and unique GEP signature; frequent 9p21 [[amplification]], [[genomic]] alterations of ''[[CIITA]]''
*Typically in [[Young adult|young adults]], [[female]] [[Predominance diagram|predominance]]; [[Mediastinum|mediastinal]] prominence with local [[Invasive (medical)|invasion]]; can involve other nodal or extranodal sites ([[kidney]] and [[liver]]); prognosis varies; [[DA-EPOCH-R]] an option
*Typically in [[Young adult|young adults]], [[female]] [[Predominance diagram|predominance]]; [[Mediastinum|mediastinal]] prominence with local [[Invasive (medical)|invasion]]; can involve other nodal or extranodal sites ([[kidney]] and [[liver]]); prognosis varies; [[DA-EPOCH-R]] an option
*
*
*'''Intravascular large B-cell lymphoma (rare)'''
 
==== '''Intravascular large B-cell lymphoma (rare)''' ====
 
*[[Lymphoma]] cells exclusively within [[Luminal|lumina]] of small or intermediate [[vessels]]; [[bone marrow]] and [[skin]] [[biopsy]] may be useful to establish [[diagnosis]]
*[[Lymphoma]] cells exclusively within [[Luminal|lumina]] of small or intermediate [[vessels]]; [[bone marrow]] and [[skin]] [[biopsy]] may be useful to establish [[diagnosis]]
*Wide [[intravascular]] [[dissemination]] ([[lung]], [[bone marrow]], [[skin]], [[CNS]], [[kidney]]), often associated with [[fever of unknown origin]] or [[Neurology|neurologic]] or [[Skin|cutaneous]] symptoms; poor [[prognosis]]
*Wide [[intravascular]] [[dissemination]] ([[lung]], [[bone marrow]], [[skin]], [[CNS]], [[kidney]]), often associated with [[fever of unknown origin]] or [[Neurology|neurologic]] or [[Skin|cutaneous]] symptoms; poor [[prognosis]]
*
*
*'''ALK-positive large B-cell lymphoma (rare)'''
 
==== '''ALK-positive large B-cell lymphoma (rare)''' ====
 
*[[ALKK|ALK]]-[[positive]] large [[B cell|B cells]], immunoblastic features and [[plasma cell]] [[phenotype]], typically [[CD20]]-negative
*[[ALKK|ALK]]-[[positive]] large [[B cell|B cells]], immunoblastic features and [[plasma cell]] [[phenotype]], typically [[CD20]]-negative
*Typically in young men with [[generalized lymphadenopathy]]; prognosis varies
*Typically in young men with [[generalized lymphadenopathy]]; prognosis varies
*
*
*'''Plasmablastic lymphoma (rare)'''
 
==== '''Plasmablastic lymphoma (rare)''' ====
 
*[[Immunoblastic Lymphadenopathy|Immunoblastic]] or [[plasmablastic]] [[B cell|B cells]], plasma-cell phenotype ([[CD138]]-[[positive]], [[CD20]]-[[Negative-sense|negative]]), often [[Epstein Barr virus|EBV]]-[[positive]]; distinguish from [[multiple myeloma]]
*[[Immunoblastic Lymphadenopathy|Immunoblastic]] or [[plasmablastic]] [[B cell|B cells]], plasma-cell phenotype ([[CD138]]-[[positive]], [[CD20]]-[[Negative-sense|negative]]), often [[Epstein Barr virus|EBV]]-[[positive]]; distinguish from [[multiple myeloma]]
*Often associated with [[HIV AIDS|HIV infection]] or [[immunosuppression]]; frequently [[Extranodal NK-T-cell lymphoma|extranodal]]; poor [[prognosis]]; consider more intensive regimens
*Often associated with [[HIV AIDS|HIV infection]] or [[immunosuppression]]; frequently [[Extranodal NK-T-cell lymphoma|extranodal]]; poor [[prognosis]]; consider more intensive regimens
*
*
*'''HHV8-positive diffuse large B-cell lymphoma (rare)'''
 
==== '''HHV8-positive diffuse large B-cell lymphoma (rare)''' ====
 
*[[HHV8]]-[[positive]] [[Immunoglobulin M|IgM]] lambda [[plasmablasts]]; often associated with [[HHV8]]-positive multicentric Castleman disease
*[[HHV8]]-[[positive]] [[Immunoglobulin M|IgM]] lambda [[plasmablasts]]; often associated with [[HHV8]]-positive multicentric Castleman disease
*Often associated with [[HIV AIDS|HIV infection]]; [[lymphadenopathy]] and [[splenomegaly]] are common; poor prognosis; no standard therapy
*Often associated with [[HIV AIDS|HIV infection]]; [[lymphadenopathy]] and [[splenomegaly]] are common; poor prognosis; no standard therapy
*
*
*'''Primary effusion lymphoma (rare)'''
 
==== '''Primary effusion lymphoma (rare)''' ====
 
*[[Immunoblastic Lymphadenopathy|Immunoblastic]] or [[plasmablastic]] B cells, [[HHV8]]-positive and usually [[Epstein Barr virus|EBV]]-[[positive]]; [[plasma-cell]] [[phenotype]] lacking usual [[B cell|B-cell]] markers; [[CD20]]-negative
*[[Immunoblastic Lymphadenopathy|Immunoblastic]] or [[plasmablastic]] B cells, [[HHV8]]-positive and usually [[Epstein Barr virus|EBV]]-[[positive]]; [[plasma-cell]] [[phenotype]] lacking usual [[B cell|B-cell]] markers; [[CD20]]-negative
*Often associated with [[HIV AIDS|HIV infection]] or [[immunosuppression]]; presents as [[Pleural cavity|pleural]], [[pericardial]], or [[Peritoneum|peritoneal]] [[Serous fluid|serous]] [[effusions]], often without detectable [[Tumor cell|tumor]] mass; poor [[prognosis]]; DA-EPOCH an option
*Often associated with [[HIV AIDS|HIV infection]] or [[immunosuppression]]; presents as [[Pleural cavity|pleural]], [[pericardial]], or [[Peritoneum|peritoneal]] [[Serous fluid|serous]] [[effusions]], often without detectable [[Tumor cell|tumor]] mass; poor [[prognosis]]; DA-EPOCH an option
*
*
*'''High-grade B-cell lymphoma'''
 
==== '''High-grade B-cell lymphoma''' ====
 
*'''High-grade B-cell lymphoma''' with ''[[MYCBPAP (gene)|MYC]]'' and ''[[BCL2-like 1|BCL2]]'' and/or ''[[BCL6]]'' rearrangements or both (doublehit or triple-hit lymphoma) (4–8% of [[Large B-cell lymphoma|large B-cell]] cases)
*'''High-grade B-cell lymphoma''' with ''[[MYCBPAP (gene)|MYC]]'' and ''[[BCL2-like 1|BCL2]]'' and/or ''[[BCL6]]'' rearrangements or both (doublehit or triple-hit lymphoma) (4–8% of [[Large B-cell lymphoma|large B-cell]] cases)
*Variable [[Morphology (biology)|morphology]], including DLBCL, [[B-cell lymphoma]] unclassifiable (with features intermediate between [[Diffuse large B cell lymphoma|DLBCL]] and [[Burkitt's lymphoma|Burkitt lymphoma]]), and blastoid features; ''[[MYCBP|MYC]]'' and ''[[BCL2-like 1|BCL2]]'' and/or ''[[BCL6]]'' rearrangements, detected by [[Fluorescence in situ hybridization|FISH]]
*Variable [[Morphology (biology)|morphology]], including DLBCL, [[B-cell lymphoma]] unclassifiable (with features intermediate between [[Diffuse large B cell lymphoma|DLBCL]] and [[Burkitt's lymphoma|Burkitt lymphoma]]), and blastoid features; ''[[MYCBP|MYC]]'' and ''[[BCL2-like 1|BCL2]]'' and/or ''[[BCL6]]'' rearrangements, detected by [[Fluorescence in situ hybridization|FISH]]
*Frequently aggressive clinical presentation; higher risk of [[CNS]] involvement; poor prognosis; consider more intensive immunochemotherapy regimens, such as DA-EPOCH-R
*Frequently aggressive clinical presentation; higher risk of [[CNS]] involvement; poor prognosis; consider more intensive immunochemotherapy regimens, such as DA-EPOCH-R
*'''High-grade B-cell lymphoma, NOS (rare)'''
 
==== '''High-grade B-cell lymphoma, NOS (rare)''' ====
 
*[[Heterogeneous]] category; often has [[morphologic features]] intermediate between [[Diffuse large B cell lymphoma|DLBCL]] and [[Burkitt's lymphoma|Burkitt lymphoma]]; lacks [[MYCBP|MYC]] and [[BCL2-like 1|BCL2]] and/or [[BCL6]] rearrangements
*[[Heterogeneous]] category; often has [[morphologic features]] intermediate between [[Diffuse large B cell lymphoma|DLBCL]] and [[Burkitt's lymphoma|Burkitt lymphoma]]; lacks [[MYCBP|MYC]] and [[BCL2-like 1|BCL2]] and/or [[BCL6]] rearrangements
*Frequently aggressive [[clinical presentation]]; increased risk of [[CNS]] involvement; poor [[prognosis]]; consider more intensive [[immunochemotherapy]] [[regimens]]
*Frequently aggressive [[clinical presentation]]; increased risk of [[CNS]] involvement; poor [[prognosis]]; consider more intensive [[immunochemotherapy]] [[regimens]]
*
*
*'''B-cell lymphoma, unclassifiable'''
 
==== '''B-cell lymphoma, unclassifiable''' ====
 
*[[B-cell lymphoma]], unclassifiable, with features intermediate between [[Diffuse large B cell lymphoma|diffuse large B-cell lymphoma]] and classic [[Hodgkin's lymphoma|Hodgkin lymphoma]] (grey-zone lymphoma) (rare)
*[[B-cell lymphoma]], unclassifiable, with features intermediate between [[Diffuse large B cell lymphoma|diffuse large B-cell lymphoma]] and classic [[Hodgkin's lymphoma|Hodgkin lymphoma]] (grey-zone lymphoma) (rare)
*Overlapping [[Morphological computation|morphologic]] or [[immunophenotypic]] features, or both, between [[Diffuse large B cell lymphoma|DLBCL]] and classic [[Hodgkin's lymphoma|Hodgkin lymphoma]]
*Overlapping [[Morphological computation|morphologic]] or [[immunophenotypic]] features, or both, between [[Diffuse large B cell lymphoma|DLBCL]] and classic [[Hodgkin's lymphoma|Hodgkin lymphoma]]

Latest revision as of 00:32, 30 April 2021

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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] Anila Hussain, MD [3]

Overview

Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on pathological and clinical features.

Classification

Classification Based on Location

Diffuse large B cell lymphoma may be classified based on location:

Classification Based on Pathological and Clinical Features

According to the updated WHO classification, diffuse large B cell lymphoma may be classified based on pathological and clinical features into the following:

Diffuse large B cell lymphoma, not otherwise specified

  • Molecular subtypes: GCB subtype, about 60%; ABC subtype, about 25–30%; unclassifiable, about 10–15%; new molecular entities recently characterized
Diagnostic features

Diffuse proliferation of medium or large lymphoid B cells typically expressing CD19, CD20, CD22, CD79a, PAX5, and surface or cytoplasmic immunoglobulin; molecular techniques (e.g., GEP) or IHC-based algorithms recommended to classify subtypes

Clinical Features and Outcome
  • Nodal presentation is the most common and 30–40% of cases are primary extranodal

Other Large B Cell Lymphoma (DLBCL) type

T-cell/histiocyte-rich large B cell lymphoma (rare)

Primary diffuse large B cell lymphoma of the central nervous system (rare)

  • Typically ABC subtype; frequent loss of HLA class I/II; frequent mutation of MYD88
  • Exclusively in CNS or intraocular region, rare systemic involvement; poor prognosis; specialized treatment with CNS-penetrating agents, with or without radiation therapy, required; targeted therapies under investigation

Primary Cutaneous DLBCL, Leg type (rare)

EBV-positive diffuse large B-cell lymphoma, NOS (rare)

EBV-positive mucocutaneous ulcer (rare)

Diffuse large B-cell lymphoma associated with chronic inflammation (rare)

Lymphomatoid granulomatosis (rare)

Large B-cell lymphoma with IRF4 rearrangement (rare)

Primary mediastinal (thymic) large B-cell lymphoma (around 6% of large B-cell cases)

Intravascular large B-cell lymphoma (rare)

ALK-positive large B-cell lymphoma (rare)

Plasmablastic lymphoma (rare)

HHV8-positive diffuse large B-cell lymphoma (rare)

Primary effusion lymphoma (rare)

High-grade B-cell lymphoma

  • High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements or both (doublehit or triple-hit lymphoma) (4–8% of large B-cell cases)
  • Variable morphology, including DLBCL, B-cell lymphoma unclassifiable (with features intermediate between DLBCL and Burkitt lymphoma), and blastoid features; MYC and BCL2 and/or BCL6 rearrangements, detected by FISH
  • Frequently aggressive clinical presentation; higher risk of CNS involvement; poor prognosis; consider more intensive immunochemotherapy regimens, such as DA-EPOCH-R

High-grade B-cell lymphoma, NOS (rare)

B-cell lymphoma, unclassifiable


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