Diffuse large B cell lymphoma classification: Difference between revisions

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==Overview==
==Overview==
Diffuse large [[B cell]] [[lymphoma]] may be classified based on location into nodal and extranodal disease and based on [[molecular]], [[genetic]], and [[immunohistochemical]] (IHC) features into more than 20 subgroups.
Diffuse large [[B cell]] [[lymphoma]] may be classified based on location into [[Nodal marginal zone lymphoma|nodal]] and [[Extranodal NK-T-cell lymphoma|extranodal]] disease and based on [[pathological]] and [[clinical]] features.


==Classification==
==Classification==
===Classification Based on Location===
===Classification Based on Location===
Diffuse large B cell lymphoma may be classified based on location:
[[Diffuse large B cell lymphoma]] may be classified based on location:
*Nodal disease
*Extranodal disease


===Classification Based on Molecular, Genetic, and Immunohistochemical Features===
*[[Nodal (protein)|Nodal]] disease
*[[Extranodal NK-T-cell lymphoma|Extranodal]] disease


According to the WHO classification, diffuse large B cell lymphoma may be classified based on [[molecular]], [[genetic]], and [[immunohistochemical]] features into the following:<ref name="WHO">{{cite book |last=Stein H, Chan JKC, Warnke RA |date=2008 |title=Diffuse large B-cell lymphoma not otherwise specified. In:Swerdlow SH, Campo E, Harris NL, et al. editors. WHO classification of tumours of haematopoietic and lymphoid tissues |location=Lyon |publisher=IARC |page=233-7 |isbn=}}</ref>
===Classification Based on Pathological and Clinical Features===
 
According to the updated [[World Health Organization|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===
===Diffuse large B cell lymphoma, not otherwise specified===
===== Morphologic subgroups=====
:* [[Centroblastic and centrocytic lymphoma|Centroblastic]] Variant
::*Most common variant, 80 percent of all cases
::*Appearance of medium-to-large-sized [[lymphocyte]]s
::**Moderate amount of [[cytoplasm]]
::**Prominently visible oval/round [[Cell nucleus|nuclei]] that contain fine [[chromatin]]
::**Two to four [[nucleoli]] within each [[nucleus]]
::*Tumor may be monomorphic composed almost entirely of centroblasts (>90%)
::*The majority of cases are polymorphic (mixture of centroblasts(<90%), immunoblasts and centrocytes)
:* Immunoblastic Variant
::*8-10 percent of all cases of DLBCL
::*Greater than 90% of its cells are [[immunoblasts]]
::**Large lymphoid cells with significant [[basophilic]] cytoplasm
::**Trapezoid shaped centrally located [[nucleolus]] with fine [[chromatin]] strands that are attached to nuclear membrane (also known as spider legs)
:* Anaplastic Variant
::*Less common variant comprising almost 3 percent of all cases of DLBCL
::*Tumor cells which appear very differently from their normal [[B cell]] counterparts
::**Very large cells with a round, oval, or polygonal shape that may resemble [[Reed-Sternberg cells]] of Hodgkin's lymphoma or [[Anaplastic]] Large cell Lymphoma
::**Pleomorphic nuclei
::*Sinusoidal Pattern
:*Other
::*Does not meet any of the above criteria


===== Immunophenotypic subgroups=====
*[[Diffuse large B cell lymphoma|Diffuse large B-cell lymphoma]], [[NOS]] (represents more than 80% of cases of [[large B-cell lymphomas]])
:* [[Germinal center]]-derived B-cell (GCB)  
 
:* Activated B-cell-like (ABC/Non GCB)
*Molecular subtypes: GCB subtype, about 60%; ABC subtype, about 25–30%; unclassifiable, about 10–15%; new molecular entities recently characterized
===== Genetic subgroups=====
 
:*''[[BCL6]]''
=====Diagnostic features=====
:*''[[BCL2-like 1|BCL2]]''
Diffuse [[proliferation]] of medium or large [[Lymphatic system|lymphoid]] [[B cell|B cells]] typically expressing [[CD19]], [[CD20]], [[CD22]], [[CD79a]], [[PAX5]], and surface or cytoplasmic [[immunoglobulin]]; [[Molecule|molecular]] [[techniques]] (e.g., [[GEP]]) or [[IHC]]-based algorithms recommended to classify subtypes
:*''[[Myc|C-MYC]]''
 
=====Immunohistochemical subgroups=====
=====Clinical Features and Outcome=====
:* [[CD5]] positive de-novo diffuse large B cell lymphoma
 
:* [[Germinal center]] B-cell like (GCB)
*The [[median]] age is 65–70 yr
:*Non-germinal center B-cell-like (non-GCB)
 
*[[Nodal marginal zone B-cell lymphoma|Nodal]] presentation is the most common and 30–40% of cases are primary [[Extranodal NK-T-cell lymphoma|extranodal]]


===Diffuse Large B Cell Lymphoma (DLBCL) sub-types<u><ref>2018 Lymphoma Research Foundation</ref></u>===
*[[prognosis]] is variable
* '''T-cell/histiocyte-rich large B cell lymphoma''':
- Consists of scattered and atypical B cells in the background of normal [[T cell|T cells]] and [[Histiocytes]]
* '''Primary diffuse large B cell lymphoma of the central nervous system (CNS)'''
- Include DLBCL in which the primary origin is in Brain or the eyes
* '''Primary Cutaneous DLBCL, Leg type'''
- Consists of Large B cells that appear as Bluish-Red or Red tumors. Despite the name , it can involve arms, legs, trunk, buttocks o anywhere on the body. These lymphomas can also spread to areas other than just the skin.
* '''EBV Positive DLBCL of Elderly'''
- Age 50 or older that are positive for [[Epstein Barr virus|EBV]]
* '''DLBCL Not Otherwise Specified (NOS)'''
- When DLBCL does not fall into any of the sub-types above, it is labelled as NOS or not otherwise specified.


-  About 25-35 percent of [[Non Hodgkin Lymphoma]] cases in Western countries are DLBCL-NOS
===Other Large B Cell Lymphoma (DLBCL) type===   


- Percentage is higher in developing countries
*'''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]]
*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)'''
*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]]
*
*'''Primary Cutaneous DLBCL, Leg type (rare)'''
*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]]
*
*'''EBV-positive diffuse large B-cell lymphoma, NOS (rare)'''


===Other lymphomas of large B cells===
*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
:* Primary mediastinal (thymic) large B-cell lymphoma
*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
:* Intravascular large [[B cell|B-cell]] [[lymphoma]]
:* DLBCL associated with chronic inflammation
:* [[Lymphomatoid granulomatosis]]  
:* ALK-positive LBCL
:* [[Plasmablastic lymphoma]]  
:* Large B-cell lymphoma arising in [[HHV8]]-associated multicentric Castleman disease
:* Primary effusion lymphoma


===Borderline cases B-cell lymphoma===
*'''EBV-positive mucocutaneous ulcer (rare)'''
:*High Grade B cell Lymphoma with MYC and BCL6 And/Or BCL4 [[Translocations]]  
*[[Polymorphism (biology)|Polymorphic]] [[infiltrate]] with frequent [[Hodgkin's lymphoma|Hodgkin]]-like cells; [[Epstein Barr virus|EBV]] is detectable in [[tumor]]
:*High Grade B cell Lymphoma, Not Otherwise Specified
*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]]
:*B cell Lymphoma, Unclassifiable with features intermixed with DLBCL and Classical [[Hodgkin Lymphoma]]
*
*'''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]]
*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)'''
*[[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
*
*'''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]]
*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)'''
*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
*
*'''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]]
*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)'''
*[[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
*
*'''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]]
*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]] [[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
*
*'''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
*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''' 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]]
*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)'''
*[[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]]
*
*'''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)
*Overlapping [[Morphological computation|morphologic]] or [[immunophenotypic]] features, or both, between [[Diffuse large B cell lymphoma|DLBCL]] and classic [[Hodgkin's lymphoma|Hodgkin lymphoma]]
*[[Male]] [[Predominance diagram|predominance]], younger age (20–40 yr); [[mediastinal]] presentation most common (80% of cases) but can occur in other sites; [[prognosis]] varies; no standard therapy, consider therapy suitable for [[Diffuse large B cell lymphoma|DLBCL]] or [[Hodgkin's lymphoma|Hodgkin lymphoma]]


==References==
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Revision as of 16:16, 16 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)
  • Few large B cells embedded in a background of T cells and histiocytes; distinguish from nodular lymphocyte-predominant Hodgkin lymphoma
  • 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)
  • 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)
  • Typically ABC subtype; frequent mutation of MYD88; distinguish from other cutaneous B-cell lymphoma
  • Typically in elderly patients and women; presents with skin nodules in lower legs; 10–15% of cases arise in other sites; poor prognosis
  • EBV-positive diffuse large B-cell lymphoma, NOS (rare)


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