HAEM4:Multicentric Castleman Disease - Revision history

Bailey.Glen: /* Cancer Sub-Classification/Subtype */

2023-11-14T16:39:50Z

Cancer Sub-Classification/Subtype

← Older revision		Revision as of 11:39, 14 November 2023
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	<blockquote class='blockedit'>{{Box-round\|title=PREVIOUS EDITION\|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents\|Table of Contents]].		<blockquote class="blockedit">{{Box-round\|title=PREVIOUS EDITION\|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents\|Table of Contents]].
	}}</blockquote>		}}</blockquote>
	==Primary Author(s)*==		==Primary Author(s)*==
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	Multicentric Castleman Disease		Multicentric Castleman Disease

	==Cancer Sub-Classification/Subtype==		==Cancer Sub-Classification / Subtype==
	None		None

	==Definition/Description of Disease==		==Definition / Description of Disease==

	Castleman disease was initially described in 1956 by Castleman ''et al'', who reported on 13 cases of localized mediastinal lymphoid proliferations in asymptomatic patients.<ref>{{Cite journal\|last=Castleman\|first=Benjamin\|last2=Iverson\|first2=Lalla\|last3=Menendez\|first3=V. Pardo\|date=1956-07\|title=Localized mediastinal lymph-node hyperplasia resembling thymoma\|url=http://dx.doi.org/10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4\|journal=Cancer\|volume=9\|issue=4\|pages=822–830\|doi=10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4\|issn=0008-543X}}</ref> It is now recognized that there are different morphologic variants (hyaline vascular, plasma cell/plasmablastic, and mixed or transitional) as well as clinical forms (unicentric and multicentric) classified under the broad clinicopathologic syndrome termed Castleman disease.<ref name=":0">{{Cite journal\|last=Chadburn\|first=Amy\|last2=Said\|first2=Jonathan\|last3=Gratzinger\|first3=Dita\|last4=Chan\|first4=John K. C.\|last5=de Jong\|first5=Daphne\|last6=Jaffe\|first6=Elaine S.\|last7=Natkunam\|first7=Yasodha\|last8=Goodlad\|first8=John R.\|date=2017-02\|title=HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms\|url=https://academic.oup.com/ajcp/article-lookup/doi/10.1093/ajcp/aqw218\|journal=American Journal of Clinical Pathology\|language=en\|volume=147\|issue=2\|pages=171–187\|doi=10.1093/ajcp/aqw218\|issn=0002-9173}}</ref> Multicentric Castleman Disease is a rare clinicopathologic entity encompassing a group of systemic polyclonal lymphoproliferative disorders. It belongs to the spectrum of HHV8-associated lymphoproliferative disorders in which there is a proliferation of morphologically benign lymphocytes, plasma cells, and vessels due to excessive production of cytokines, IL6 features prominently amongst these.<ref name=":1">{{Cite journal\|last=Swerdlow\|first=Steven H.\|last2=Campo\|first2=Elias\|last3=Pileri\|first3=Stefano A.\|last4=Harris\|first4=Nancy Lee\|last5=Stein\|first5=Harald\|last6=Siebert\|first6=Reiner\|last7=Advani\|first7=Ranjana\|last8=Ghielmini\|first8=Michele\|last9=Salles\|first9=Gilles A.\|date=2016-05-19\|title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms\|url=http://dx.doi.org/10.1182/blood-2016-01-643569\|journal=Blood\|volume=127\|issue=20\|pages=2375–2390\|doi=10.1182/blood-2016-01-643569\|issn=0006-4971}}</ref>Multicentric Castleman disease is idiopathic in HIV-negative and HHV8-negative patients.		Castleman disease was initially described in 1956 by Castleman ''et al'', who reported on 13 cases of localized mediastinal lymphoid proliferations in asymptomatic patients.<ref>{{Cite journal\|last=Castleman\|first=Benjamin\|last2=Iverson\|first2=Lalla\|last3=Menendez\|first3=V. Pardo\|date=1956-07\|title=Localized mediastinal lymph-node hyperplasia resembling thymoma\|url=http://dx.doi.org/10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4\|journal=Cancer\|volume=9\|issue=4\|pages=822–830\|doi=10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4\|issn=0008-543X}}</ref> It is now recognized that there are different morphologic variants (hyaline vascular, plasma cell/plasmablastic, and mixed or transitional) as well as clinical forms (unicentric and multicentric) classified under the broad clinicopathologic syndrome termed Castleman disease.<ref name=":0">{{Cite journal\|last=Chadburn\|first=Amy\|last2=Said\|first2=Jonathan\|last3=Gratzinger\|first3=Dita\|last4=Chan\|first4=John K. C.\|last5=de Jong\|first5=Daphne\|last6=Jaffe\|first6=Elaine S.\|last7=Natkunam\|first7=Yasodha\|last8=Goodlad\|first8=John R.\|date=2017-02\|title=HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms\|url=https://academic.oup.com/ajcp/article-lookup/doi/10.1093/ajcp/aqw218\|journal=American Journal of Clinical Pathology\|language=en\|volume=147\|issue=2\|pages=171–187\|doi=10.1093/ajcp/aqw218\|issn=0002-9173}}</ref> Multicentric Castleman Disease is a rare clinicopathologic entity encompassing a group of systemic polyclonal lymphoproliferative disorders. It belongs to the spectrum of HHV8-associated lymphoproliferative disorders in which there is a proliferation of morphologically benign lymphocytes, plasma cells, and vessels due to excessive production of cytokines, IL6 features prominently amongst these.<ref name=":1">{{Cite journal\|last=Swerdlow\|first=Steven H.\|last2=Campo\|first2=Elias\|last3=Pileri\|first3=Stefano A.\|last4=Harris\|first4=Nancy Lee\|last5=Stein\|first5=Harald\|last6=Siebert\|first6=Reiner\|last7=Advani\|first7=Ranjana\|last8=Ghielmini\|first8=Michele\|last9=Salles\|first9=Gilles A.\|date=2016-05-19\|title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms\|url=http://dx.doi.org/10.1182/blood-2016-01-643569\|journal=Blood\|volume=127\|issue=20\|pages=2375–2390\|doi=10.1182/blood-2016-01-643569\|issn=0006-4971}}</ref>Multicentric Castleman disease is idiopathic in HIV-negative and HHV8-negative patients.

	==Synonyms/Terminology==		==Synonyms / Terminology==

	Angio follicular lymph-node hyperplasia		Angio follicular lymph-node hyperplasia
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	Giant node hyperplasia		Giant node hyperplasia

	==Epidemiology/Prevalence==		==Epidemiology / Prevalence==

	HHV8 Positive Multicentric Castleman Disease occurs in immunosuppressed patients across all ethnic groups, particularly in association with HIV/AIDS. <ref name=":1" /> Immunocompetent individuals may be affected with the disease in HHV8 endemic areas such as sub-Saharan Africa and Mediterranean countries. In cases where HIV was acquired via sexual transmission, there is a strong association with the development of HHV8-positive MCD, men are predominantly affected.		HHV8 Positive Multicentric Castleman Disease occurs in immunosuppressed patients across all ethnic groups, particularly in association with HIV/AIDS. <ref name=":1" /> Immunocompetent individuals may be affected with the disease in HHV8 endemic areas such as sub-Saharan Africa and Mediterranean countries. In cases where HIV was acquired via sexual transmission, there is a strong association with the development of HHV8-positive MCD, men are predominantly affected.
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	==References==		==References==
	<references />		<references />
	[[Category:HAEM4]] [[Category:DISEASE]]		[[Category:HAEM4]]
			[[Category:DISEASE]]

Bailey.Glen at 18:54, 3 November 2023

2023-11-03T18:54:09Z

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 ==Primary Author(s)*==
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 ==References==
 <references />

Bailey.Glen: Created page with "==Primary Author(s)*== Sudha Arumugam, MD TOC ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."

2023-11-03T18:17:35Z

Created page with "==Primary Author(s)*== Sudha Arumugam, MD __TOC__ ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."

New page

==Primary Author(s)*==

Sudha Arumugam, MD

__TOC__

==Cancer Category/Type==

Multicentric Castleman Disease

==Cancer Sub-Classification/Subtype==
None

==Definition/Description of Disease==

Castleman disease was initially described in 1956 by Castleman ''et al'', who reported on 13 cases of localized mediastinal lymphoid proliferations in asymptomatic patients.<ref>{{Cite journal|last=Castleman|first=Benjamin|last2=Iverson|first2=Lalla|last3=Menendez|first3=V. Pardo|date=1956-07|title=Localized mediastinal lymph-node hyperplasia resembling thymoma|url=http://dx.doi.org/10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4|journal=Cancer|volume=9|issue=4|pages=822–830|doi=10.1002/1097-0142(195607/08)9:4<822::aid-cncr2820090430>3.0.co;2-4|issn=0008-543X}}</ref> It is now recognized that there are different morphologic variants (hyaline vascular, plasma cell/plasmablastic, and mixed or transitional) as well as clinical forms (unicentric and multicentric) classified under the broad clinicopathologic syndrome termed Castleman disease.<ref name=":0">{{Cite journal|last=Chadburn|first=Amy|last2=Said|first2=Jonathan|last3=Gratzinger|first3=Dita|last4=Chan|first4=John K. C.|last5=de Jong|first5=Daphne|last6=Jaffe|first6=Elaine S.|last7=Natkunam|first7=Yasodha|last8=Goodlad|first8=John R.|date=2017-02|title=HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms|url=https://academic.oup.com/ajcp/article-lookup/doi/10.1093/ajcp/aqw218|journal=American Journal of Clinical Pathology|language=en|volume=147|issue=2|pages=171–187|doi=10.1093/ajcp/aqw218|issn=0002-9173}}</ref> Multicentric Castleman Disease is a rare clinicopathologic entity encompassing a group of systemic polyclonal lymphoproliferative disorders. It belongs to the spectrum of HHV8-associated lymphoproliferative disorders in which there is a proliferation of morphologically benign lymphocytes, plasma cells, and vessels due to excessive production of cytokines, IL6 features prominently amongst these.<ref name=":1">{{Cite journal|last=Swerdlow|first=Steven H.|last2=Campo|first2=Elias|last3=Pileri|first3=Stefano A.|last4=Harris|first4=Nancy Lee|last5=Stein|first5=Harald|last6=Siebert|first6=Reiner|last7=Advani|first7=Ranjana|last8=Ghielmini|first8=Michele|last9=Salles|first9=Gilles A.|date=2016-05-19|title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms|url=http://dx.doi.org/10.1182/blood-2016-01-643569|journal=Blood|volume=127|issue=20|pages=2375–2390|doi=10.1182/blood-2016-01-643569|issn=0006-4971}}</ref>Multicentric Castleman disease is idiopathic in HIV-negative and HHV8-negative patients.

==Synonyms/Terminology==

Angio follicular lymph-node hyperplasia

Giant node hyperplasia

==Epidemiology/Prevalence==

HHV8 Positive Multicentric Castleman Disease occurs in immunosuppressed patients across all ethnic groups, particularly in association with HIV/AIDS. <ref name=":1" /> Immunocompetent individuals may be affected with the disease in HHV8 endemic areas such as sub-Saharan Africa and Mediterranean countries. In cases where HIV was acquired via sexual transmission, there is a strong association with the development of HHV8-positive MCD, men are predominantly affected.

==Clinical Features==

<br />
{| class="wikitable"
|'''Signs and Symptoms'''
|Progressive lymphadenopathy
Splenomegaly or hepatosplenomegaly

Fever

Night sweats

Fatigue

Weight loss

Respiratory symptoms

Coincident Kaposi Sarcoma or HHV8-positive diffuse large B-cell lymphoma

Skin rash
|-
|'''Laboratory Findings'''
|Anemia
Thrombocytopenia

Hypoalbuminemia

Hypogammaglobinemia

Elevated C-reactive protein
|}

==Sites of Involvement==

Multicentric Castleman Disease affects multiple lymph node sites, predominantly in the cervical region and commonly involves the spleen.<ref name=":2">Balakrishna J. Castleman disease. PathologyOutlines.com website.<nowiki>https://www.pathologyoutlines.com/topic/lymphnodescastleman.html</nowiki>. Accessed November 24th, 2021. </ref>

==Morphologic Features==

HHV8-infected plasmablasts are the characteristic features of HHV8 Multicentric Castleman disease.<ref name=":2" /> Plasmablasts are present in the lymph node and spleen.<ref name=":0" /> The B cell follicles of lymph nodes shows varying degree of hyalinization and involution of germinal centers with prominent mantle zones that may intrude and efface the germinal centers.<ref name=":1" /> Follicles may show onion skinning or widened concentric rings of mantle zone lymphocytes along with prominent penetrating venules, these findings are typical of Castleman disease.<ref name=":1" /> Variable numbers of medium to large plasmablasts with amphophilic cytoplasm and eccentrically placed nuclei can be found among the mantle zone cells and adjacent interfollicular regions.<ref name=":1" /> Sheets of mature plasma cells may be seen expanding the interfollicular region, some such cells may display cytoplasmic inclusions (Russell bodies) or crystalline forms.<ref name=":1" /> With disease progression, the plasmablasts increase in number and may coalesce to form clusters.<ref name=":1" /> If the disease proceeds to become HHV8-positive diffuse large B-cell lymphoma, NOS these clusters may expand clonally to form sheets of lymphoma cells that efface the normal follicular architecture.<ref name=":1" /> Plasmablastic aggregates that develop during disease progression may be oligoclonal or monoclonal.

==Immunophenotype==

{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||HHV 8 LANA 1, strong c IgM expression with lambda light chain restriction, CD20+/-, CD 79a -/+ <ref name=":1" />
|-
|Positive (subset)||Viral IL-6 <ref name=":1" />
|-
|Negative (universal)||CD 138, PAX 5, CD38-/+, CD27, EBV encoded small RNA <ref name=":1" />
|-
|Negative (subset)||None
|}

==Chromosomal Rearrangements (Gene Fusions)==

No known recurrent abnormalities
==Individual Region Genomic Gain/Loss/LOH==

No known recurrent abnormalities
==Characteristic Chromosomal Patterns==

No known recurrent abnormalities
==Gene Mutations (SNV/INDEL)==

No known recurrent abnormalities

==Epigenomic Alterations==

No known recurrent abnormalities

==Genes and Main Pathways Involved==

No known recurrent abnormalities
==Genetic Diagnostic Testing Methods==

No known recurrent abnormalities

==Familial Forms==

Given there are no recurrent genetic abnormalities associated with MCD, no method can be recommended at present.

==Additional Information==

Put your text here

==Links==

None

==References==
<references />

HAEM4:Multicentric Castleman Disease - Revision history

Bailey.Glen: /* Cancer Sub-Classification/Subtype */

Bailey.Glen at 18:54, 3 November 2023

Bailey.Glen: Created page with "==Primary Author(s)*== Sudha Arumugam, MD __TOC__ ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."

Bailey.Glen: Created page with "==Primary Author(s)*== Sudha Arumugam, MD TOC ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."