Bailey.Glen at 18:58, 3 November 2023

2023-11-03T18:58:06Z

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 {{Under Construction}}
 ==Primary Author(s)*==
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 <nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.
 <references />

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Patricia V. Hernandez, M.D., Washington University School of Medicine TOC ==Cancer Category/Type== Hodgkin's lymphoma ==..."

2023-11-03T18:23:45Z

Created page with "{{Under Construction}} ==Primary Author(s)*== Patricia V. Hernandez, M.D., Washington University School of Medicine __TOC__ ==Cancer Category/Type== Hodgkin's lymphoma ==..."

New page

{{Under Construction}}
==Primary Author(s)*==

Patricia V. Hernandez, M.D., Washington University School of Medicine

__TOC__

==Cancer Category/Type==

Hodgkin's lymphoma

==Cancer Sub-Classification / Subtype==

N/A

==Definition / Description of Disease==

Variant of classical Hodgkin's lymphoma rich in lymphocytes, with expanded mantle zones, atrophic germinal centers, small number of Reed-Stenberg cells and classical Hodgkin's lymphoma immonophenotype <ref name=":2">{{Cite journal|last=Nam-Cha|first=Syong H.|last2=Montes-Moreno|first2=Santiago|last3=Salcedo|first3=Maria T.|last4=Sanjuan|first4=Josefina|last5=Garcia|first5=Juan F.|last6=Piris|first6=Miguel A.|date=2009-08|title=Lymphocyte-rich classical Hodgkin's lymphoma: distinctive tumor and microenvironment markers|url=https://pubmed.ncbi.nlm.nih.gov/19465900|journal=Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc|volume=22|issue=8|pages=1006–1015|doi=10.1038/modpathol.2009.54|issn=1530-0285|pmid=19465900}}</ref><ref name=":3" />.

==Synonyms / Terminology==

N/A

==Epidemiology / Prevalence==

It was the last subtype of classical Hodgkin lymphoma to be described and presents a low frequency, accounting for 3-5% of classical Hodgkin lymphomas <ref name=":1">{{Cite journal|last=Jiang|first=Manli|last2=Bennani|first2=N. Nora|last3=Feldman|first3=Andrew L.|date=2017-03|title=Lymphoma classification update: T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms|url=https://pubmed.ncbi.nlm.nih.gov/28133975|journal=Expert Review of Hematology|volume=10|issue=3|pages=239–249|doi=10.1080/17474086.2017.1281122|issn=1747-4094|pmc=5514564|pmid=28133975}}</ref>. Because of its rarity, the literature is limited on this type of lymphoma.

==Clinical Features==

The main clinical features are listed below <ref name=":3">{{Cite journal|last=Diehl|first=V.|last2=Sextro|first2=M.|last3=Franklin|first3=J.|last4=Hansmann|first4=M. L.|last5=Harris|first5=N.|last6=Jaffe|first6=E.|last7=Poppema|first7=S.|last8=Harris|first8=M.|last9=Franssila|first9=K.|date=1999-03|title=Clinical presentation, course, and prognostic factors in lymphocyte-predominant Hodgkin's disease and lymphocyte-rich classical Hodgkin's disease: report from the European Task Force on Lymphoma Project on Lymphocyte-Predominant Hodgkin's Disease|url=https://pubmed.ncbi.nlm.nih.gov/10071266|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=17|issue=3|pages=776–783|doi=10.1200/JCO.1999.17.3.776|issn=0732-183X|pmid=10071266}}</ref><ref name=":0" /><ref>{{Cite journal|last=Shimabukuro-Vornhagen|first=Alexander|last2=Haverkamp|first2=Heinz|last3=Engert|first3=Andreas|last4=Balleisen|first4=Leopold|last5=Majunke|first5=Peter|last6=Heil|first6=Günther|last7=Eich|first7=Hans Theodor|last8=Stein|first8=Harald|last9=Diehl|first9=Volker|date=2005-08-20|title=Lymphocyte-rich classical Hodgkin's lymphoma: clinical presentation and treatment outcome in 100 patients treated within German Hodgkin's Study Group trials|url=https://pubmed.ncbi.nlm.nih.gov/16009944|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=23|issue=24|pages=5739–5745|doi=10.1200/JCO.2005.17.970|issn=0732-183X|pmid=16009944}}</ref>.
{| class="wikitable"
|'''Signs and Symptoms'''
|Older in age
Presentation at early stages are common

Infrequent B symptoms (weight loss, fever, night sweats)

Infrequent mediastinal involvement and bulky disease

Usually good prognosis
|-
|'''Laboratory Findings'''
|There is no characteristic finding in this condition. It can present with anemia, lymphocytopenia as a common condition of Hodgkin's lymphoma
|}

==Sites of Involvement==

Nodal disease with involvement of cervical lymph nodes. Mediastinal mass are rarely seen.

==Morphologic Features==

Lymphocyte-rich subtype presents intermediate characteristics between those of classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma <ref name=":2" /><ref name=":1" />. The main feature is the predominance of small-lymphocytes in the background resembling nodular sclerosis classical Hodgkin Lymphoma,<ref name=":4">{{Cite journal|last=Wang|first=Hao-Wei|last2=Balakrishna|first2=Jayalakshmi P.|last3=Pittaluga|first3=Stefania|last4=Jaffe|first4=Elaine S.|date=2019-01|title=Diagnosis of Hodgkin lymphoma in the modern era|url=https://pubmed.ncbi.nlm.nih.gov/30407610|journal=British Journal of Haematology|volume=184|issue=1|pages=45–59|doi=10.1111/bjh.15614|issn=1365-2141|pmc=6310079|pmid=30407610}}</ref> but with T-cell populations surrounding the germinal centers <ref name=":2" />. Classic Reed Sternberg cells are found adjacent to germinal centers in approximately 89.5% of cases although they account for only a small fraction of the cells <ref name=":0">{{Cite journal|last=Anagnostopoulos|first=I.|last2=Hansmann|first2=M. L.|last3=Franssila|first3=K.|last4=Harris|first4=M.|last5=Harris|first5=N. L.|last6=Jaffe|first6=E. S.|last7=Han|first7=J.|last8=van Krieken|first8=J. M.|last9=Poppema|first9=S.|date=2000-09-01|title=European Task Force on Lymphoma project on lymphocyte predominance Hodgkin disease: histologic and immunohistologic analysis of submitted cases reveals 2 types of Hodgkin disease with a nodular growth pattern and abundant lymphocytes|url=https://pubmed.ncbi.nlm.nih.gov/10961891|journal=Blood|volume=96|issue=5|pages=1889–1899|issn=0006-4971|pmid=10961891}}</ref>. There are three growth patterns, the most frequent is the nodular pattern, with intact or atrophic germinal centers composed of small lymphoid cells displaying round nuclei <ref name=":0" /> . Other growth patterns are interfollicular, composed of neoplastic cells forming small nodules with expansion to interfollicular areas and infiltration to adjacent mantle zones, and more rarely a diffuse growth pattern in which a germinal center is not seen <ref name=":0" />.

==Immunophenotype==

Immunophenotype of lymphoma-rich classical Hodgkin lymphoma is shown in the table below <ref name=":2" /><ref name=":0" />.

{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||CD30 <ref name=":2" />, MUM-1 <ref name=":2" />, TRA-1 <ref name=":2" />
|-
|Positive (subset)||CD15 (56%) <ref name=":2" />, EBER (31%) <ref name=":2" />, CD20 (31%) <ref name=":2" />, CD79a (13%) <ref name=":0" />, OCT.1 (50%) <ref name=":2" />, OCT.2 (56%) <ref name=":2" />, BOB.1 (62%) <ref name=":2" />, Pax-5 (94%) <ref name=":2" />, KLHL6 (69%) <ref name=":2" />, P-50 (73%) <ref name=":2" />
|-
|Negative (universal)||GCET-1 <ref name=":2" />, J-chain <ref name=":0" />
|-
|Negative (subset)||CD20 (68%) <ref name=":0" />, EBER (53%) <ref name=":0" />, BCL6 (64%) <ref name=":2" />, C-REL (75%) <ref name=":2" />, REL-B (88%) <ref name=":2" />, IgD (94%) <ref name=":2" />, Blimp-1 (25%) <ref name=":2" />
|}

==Chromosomal Rearrangements (Gene Fusions)==

No characteristic chromosomal rearrangements have been reported for lymphocyte-rich classical Hodgkin's lymphoma.

{| class="wikitable sortable"
|-
!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)
EXAMPLE 30% (add reference)
|Yes
|No
|Yes
|EXAMPLE

The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).
|}

==Individual Region Genomic Gain/Loss/LOH==

There is no typical findings for lymphocyte-rich classical Hodgkin's lymphoma, the main features are also seen in other classical Hodgkin's lymphoma subtypes.

{| class="wikitable sortable"
|-
!Chr #!!Gain / Loss / Amp / LOH!!Minimal Region Genomic Coordinates [Genome Build]!!Minimal Region Cytoband
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|2p <ref name=":4" /><ref name=":5">{{Cite journal|last=Steidl|first=Christian|last2=Telenius|first2=Adele|last3=Shah|first3=Sohrab P.|last4=Farinha|first4=Pedro|last5=Barclay|first5=Lorena|last6=Boyle|first6=Merrill|last7=Connors|first7=Joseph M.|last8=Horsman|first8=Douglas E.|last9=Gascoyne|first9=Randy D.|date=2010-07-22|title=Genome-wide copy number analysis of Hodgkin Reed-Sternberg cells identifies recurrent imbalances with correlations to treatment outcome|url=https://pubmed.ncbi.nlm.nih.gov/20339089|journal=Blood|volume=116|issue=3|pages=418–427|doi=10.1182/blood-2009-12-257345|issn=1528-0020|pmid=20339089}}</ref>
|Gain
|
|chr2
|Unknown
|Unknown
|Unknown
|It can result in ''REL'' (2p16), ''CD40'' (20q13) <ref name=":4" />
|-
|9p <ref name=":4" /><ref name=":5" />
|Gain
|
|chr9
|Unknown
|Unknown
|Unknown
|It can result in ''JAK2'', ''CD274'' (''PDL1'') and ''PDCD1LG2'' (''PDL2'') mutations. The amplification of 9p24.1 is critical to CD274/PDCD1LG2 gain of function<ref name=":4" />
|-
|17q <ref name=":4" /><ref name=":5" />
|Gain
|
|chr17
|Unknown
|Unknown
|Unknown
|it can result in ''MAP3K14'' (17q21) mutation<ref name=":4" />
|-
|19q <ref name=":4" /><ref name=":5" />
|Gain
|
|chr19
|Unknown
|Unknown
|Unknown
|It can result in ''RELB'' (19q13) mutation<ref name=":4" />
|-
|20q <ref name=":4" /><ref name=":5" />
|Gain
|
|chr20
|Unknown
|Unknown
|Unknown
|''CD40'' is the lesion of 20q13<ref name=":4" />
|-
|6q <ref name=":4" /><ref name=":5" />
|Loss
|
|chr6
|Unknown
|Unknown
|Unknown
|
|-
|13q <ref name=":4" /><ref name=":5" />
|Loss
|
|chr13
|Unknown
|Unknown
|Unknown
|
|}
==Characteristic Chromosomal Patterns==

No characteristic chromosomal patterns have been reported for lymphocyte-rich classical Hodgkin's lymphoma.

{| class="wikitable sortable"
|-
!Chromosomal Pattern
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|EXAMPLE

Co-deletion of 1p and 18q
|Yes
|No
|No
|EXAMPLE:

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
|}
==Gene Mutations (SNV/INDEL)==

No characteristic gene mutations have been reported for lymphocyte-rich classical Hodgkin's lymphoma.

{| class="wikitable sortable"
|-
!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC / TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
!'''Diagnostic Significance (Yes, No or Unknown)'''
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|EXAMPLE: TP53; Variable LOF mutations

EXAMPLE:

EGFR; Exon 20 mutations

EXAMPLE: BRAF; Activating mutations
|EXAMPLE: TSG
|EXAMPLE: 20% (COSMIC)

EXAMPLE: 30% (add Reference)
|EXAMPLE: IDH1 R123H
|EXAMPLE: EGFR amplification
|
|
|
|EXAMPLE: Excludes hairy cell leukemia (HCL) (add reference).
<br />
|}
Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

==Epigenomic Alterations==

N/A

==Genes and Main Pathways Involved==

N/A
{| class="wikitable sortable"
|-
!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
|-
|EXAMPLE: BRAF and MAP2K1; Activating mutations
|EXAMPLE: MAPK signaling
|EXAMPLE: Increased cell growth and proliferation
|-
|EXAMPLE: CDKN2A; Inactivating mutations
|EXAMPLE: Cell cycle regulation
|EXAMPLE: Unregulated cell division
|-
|EXAMPLE: KMT2C and ARID1A; Inactivating mutations
|EXAMPLE: Histone modification, chromatin remodeling
|EXAMPLE: Abnormal gene expression program
|-
|''REL'' (2p16), ''RELB'' (19q13), ''CD40'' (20q13) and ''MAP3K14'' (17q21)
|nuclear factor (NF)-κB signalling
|
|-
|''JAK2'' amplification
|PD-L1 protein expression/ JAK/STAT pathway
|
|}
==Genetic Diagnostic Testing Methods==

N/A

==Familial Forms==

N/A

==Additional Information==

N/A

==Links==

Put your text placeholder here (use "Link" icon at top of page)

==References==
<references />
(use "Cite" icon at top of page)
===EXAMPLE Book===

#Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171.

==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.
<references />

HAEM4:Lymphocyte-Rich Classic Hodgkin Lymphoma - Revision history

Bailey.Glen at 18:58, 3 November 2023

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Patricia V. Hernandez, M.D., Washington University School of Medicine __TOC__ ==Cancer Category/Type== Hodgkin's lymphoma ==..."

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Patricia V. Hernandez, M.D., Washington University School of Medicine TOC ==Cancer Category/Type== Hodgkin's lymphoma ==..."