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 features into more than 20 subgroups. | 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. | ||
==Classification== | ==Classification== | ||
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===Classification Based on Molecular, Genetic, and Immunohistochemical Features=== | ===Classification Based on Molecular, Genetic, and Immunohistochemical Features=== | ||
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> | 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> | ||
=== | ===Diffuse large B cell lymphoma, not otherwise specified=== | ||
===== | ===== Morphologic subgroups===== | ||
:* Centroblastic Variant | :* Centroblastic Variant | ||
::*Most common variant, 80 percent of all cases | ::*Most common variant, 80 percent of all cases | ||
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:* Immunoblastic Variant | :* Immunoblastic Variant | ||
::*8-10 percent of all cases of DLBCL | ::*8-10 percent of all cases of DLBCL | ||
::*Greater than 90% of its cells are immunoblasts | ::*Greater than 90% of its cells are [[immunoblasts]] | ||
::**Large lymphoid cells with Significant [[basophilic]] cytoplasm | ::**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) | ::**Trapezoid shaped centrally located [[nucleolus]] with fine [[chromatin]] strands that are attached to nuclear membrane(also known as spider legs) | ||
:* Anaplastic Variant | :* Anaplastic Variant | ||
::*Less common variant comprising almost 3 percent of all cases of DLBCL | ::*Less common variant comprising almost 3 percent of all cases of DLBCL | ||
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::*Does not meet any of the above criteria | ::*Does not meet any of the above criteria | ||
===== | ===== Immunophenotypic subgroups===== | ||
:* Germinal center-derived B-cell (GCB) | :* Germinal center-derived B-cell (GCB) | ||
:* Activated B-cell-like (ABC/Non GCB) | :* Activated B-cell-like (ABC/Non GCB) | ||
===== | ===== Genetic subgroups===== | ||
:*''BCL6'' | :*''BCL6'' | ||
:*''BCL2'' | :*''BCL2'' | ||
:*''C-MYC'' | :*''C-MYC'' | ||
:*Other | :*Other | ||
===== | =====Immunohistochemical subgroups===== | ||
:* CD5-positive de-novo diffuse large B cell lymphoma | :* CD5-positive de-novo diffuse large B cell lymphoma | ||
:* Germinal center B-cell like (GCB) | :* Germinal center B-cell like (GCB) | ||
:*Non-germinal center B-cell-like (non-GCB) | :*Non-germinal center B-cell-like (non-GCB) | ||
=== | ===Diffuse Large B Cell Lymphoma (DLBCL) sub-types<u><ref>2018 Lymphoma Research Foundation</ref></u>=== | ||
* '''T-cell/histiocyte-rich large B cell lymphoma''': | |||
- Consists of scattered and atypical B cells in the background of normal T cells and Histiocytes | - Consists of scattered and atypical B cells in the background of normal 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 | - 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. | - 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 EBV | - Age 50 or older that are positive for 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. | - When DLBCL does not fall into any of the sub-types above, it is labelled as NOS or not otherwise specified. | ||
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- Percentage is higher in developing countries | - Percentage is higher in developing countries | ||
=== | ===Other lymphomas of large B cells=== | ||
:* Primary mediastinal (thymic) large B-cell lymphoma | :* Primary mediastinal (thymic) large B-cell lymphoma | ||
:* Intravascular large B-cell lymphoma | :* Intravascular large B-cell lymphoma | ||
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:* Primary effusion lymphoma | :* Primary effusion lymphoma | ||
=== | ===Borderline cases B-cell lymphoma=== | ||
:*High Grade B cell Lymphoma with MYC and BCL6 And/Or BCL4 Translocations | :*High Grade B cell Lymphoma with MYC and BCL6 And/Or BCL4 Translocations | ||
:*High Grade B cell Lymphoma, Not Otherwise Specified | :*High Grade B cell Lymphoma, Not Otherwise Specified | ||
Revision as of 02:19, 13 July 2018
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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 molecular, genetic, and immunohistochemical (IHC) features into more than 20 subgroups.
Classification
Classification 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
According to the WHO classification, diffuse large B cell lymphoma may be classified based on molecular, genetic, and immunohistochemical features into the following:[1]
Diffuse large B cell lymphoma, not otherwise specified
Morphologic subgroups
- Centroblastic Variant
- Most common variant, 80 percent of all cases
- Appearance of medium-to-large-sized lymphocytes
- 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
- Germinal center-derived B-cell (GCB)
- Activated B-cell-like (ABC/Non GCB)
Genetic subgroups
- BCL6
- BCL2
- C-MYC
- Other
Immunohistochemical subgroups
- CD5-positive de-novo diffuse large B cell lymphoma
- Germinal center B-cell like (GCB)
- Non-germinal center B-cell-like (non-GCB)
Diffuse Large B Cell Lymphoma (DLBCL) sub-types[2]
- T-cell/histiocyte-rich large B cell lymphoma:
- Consists of scattered and atypical B cells in the background of normal 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 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
- Percentage is higher in developing countries
Other lymphomas of large B cells
- Primary mediastinal (thymic) large B-cell lymphoma
- Intravascular large 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
- High Grade B cell Lymphoma with MYC and BCL6 And/Or BCL4 Translocations
- High Grade B cell Lymphoma, Not Otherwise Specified
- B cell Lymphoma, Unclassifiable with features intermixed with DLBCL and Classical Hodgkin Lymphoma