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| |Subtype(s) | | |Subtype(s) |
| |Classic Hodgkin lymphoma | | |Classic Hodgkin lymphoma |
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| ==Definition / Description of Disease==
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| 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" />.
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| ==Synonyms / Terminology==
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| N/A
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| ==Epidemiology / Prevalence==
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| 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.
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| ==Clinical Features==
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| 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>.
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| {| class="wikitable"
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| |'''Signs and Symptoms'''
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| |Older in age
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| Presentation at early stages are common
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| Infrequent B symptoms (weight loss, fever, night sweats)
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| Infrequent mediastinal involvement and bulky disease
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| Usually good prognosis
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| |-
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| |'''Laboratory Findings'''
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| |There is no characteristic finding in this condition. It can present with anemia, lymphocytopenia as a common condition of Hodgkin's lymphoma
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| |}
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| ==Sites of Involvement==
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| Nodal disease with involvement of cervical lymph nodes. Mediastinal mass are rarely seen.
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| ==Morphologic Features==
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| 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" />.
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| ==Immunophenotype==
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| Immunophenotype of lymphoma-rich classical Hodgkin lymphoma is shown in the table below <ref name=":2" /><ref name=":0" />.
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| {| class="wikitable sortable"
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| |-
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| !Finding!!Marker
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| |-
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| |Positive (universal)||CD30 <ref name=":2" />, MUM-1 <ref name=":2" />, TRA-1 <ref name=":2" />
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| |-
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| |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" />
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| |-
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| |Negative (universal)||GCET-1 <ref name=":2" />, J-chain <ref name=":0" />
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| |-
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| |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" />
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| |} | | |} |
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| !Notes | | !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> | | |2p <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><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 | | |Gain |
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