https://test.ccga.io/index.php?action=history&feed=atom&title=HAEM4Backup%3AMulticentric_Castleman_DiseaseHAEM4Backup:Multicentric Castleman Disease - Revision history2026-04-30T21:45:16ZRevision history for this page on the wikiMediaWiki 1.43.5https://test.ccga.io/index.php?title=HAEM4Backup:Multicentric_Castleman_Disease&diff=12269&oldid=prevBailey.Glen: Created page with "==Primary Author(s)*== Sudha Arumugam, MD __TOC__ ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."2023-11-03T17:46:04Z<p>Created page with "==Primary Author(s)*== Sudha Arumugam, MD __TOC__ ==Cancer Category/Type== Multicentric Castleman Disease ==Cancer Sub-Classification/Subtype== None ==Definition/Descrip..."</p>
<p><b>New page</b></p><div>==Primary Author(s)*==<br />
<br />
Sudha Arumugam, MD<br />
<br />
__TOC__<br />
<br />
==Cancer Category/Type==<br />
<br />
Multicentric Castleman Disease<br />
<br />
==Cancer Sub-Classification/Subtype==<br />
None<br />
<br />
==Definition/Description of Disease==<br />
<br />
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.<br />
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==Synonyms/Terminology==<br />
<br />
Angio follicular lymph-node hyperplasia <br />
<br />
Giant node hyperplasia <br />
<br />
==Epidemiology/Prevalence==<br />
<br />
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.<br />
<br />
==Clinical Features==<br />
<br />
<br /><br />
{| class="wikitable"<br />
|'''Signs and Symptoms'''<br />
|Progressive lymphadenopathy<br />
Splenomegaly or hepatosplenomegaly<br />
<br />
Fever<br />
<br />
Night sweats<br />
<br />
Fatigue<br />
<br />
Weight loss<br />
<br />
Respiratory symptoms <br />
<br />
Coincident Kaposi Sarcoma or HHV8-positive diffuse large B-cell lymphoma<br />
<br />
Skin rash<br />
|-<br />
|'''Laboratory Findings'''<br />
|Anemia<br />
Thrombocytopenia<br />
<br />
Hypoalbuminemia<br />
<br />
Hypogammaglobinemia<br />
<br />
Elevated C-reactive protein<br />
|}<br />
<br />
==Sites of Involvement==<br />
<br />
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><br />
<br />
==Morphologic Features==<br />
<br />
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. <br />
<br />
==Immunophenotype==<br />
<br />
{| class="wikitable sortable"<br />
|-<br />
!Finding!!Marker<br />
|-<br />
|Positive (universal)||HHV 8 LANA 1, strong c IgM expression with lambda light chain restriction, CD20+/-, CD 79a -/+ <ref name=":1" /><br />
|-<br />
|Positive (subset)||Viral IL-6 <ref name=":1" /><br />
|-<br />
|Negative (universal)||CD 138, PAX 5, CD38-/+, CD27, EBV encoded small RNA <ref name=":1" /><br />
|-<br />
|Negative (subset)||None<br />
|}<br />
<br />
==Chromosomal Rearrangements (Gene Fusions)==<br />
<br />
No known recurrent abnormalities <br />
==Individual Region Genomic Gain/Loss/LOH==<br />
<br />
No known recurrent abnormalities <br />
==Characteristic Chromosomal Patterns==<br />
<br />
No known recurrent abnormalities <br />
==Gene Mutations (SNV/INDEL)==<br />
<br />
No known recurrent abnormalities <br />
<br />
==Epigenomic Alterations==<br />
<br />
No known recurrent abnormalities <br />
<br />
==Genes and Main Pathways Involved==<br />
<br />
No known recurrent abnormalities <br />
==Genetic Diagnostic Testing Methods==<br />
<br />
No known recurrent abnormalities<br />
<br />
==Familial Forms==<br />
<br />
Given there are no recurrent genetic abnormalities associated with MCD, no method can be recommended at present.<br />
<br />
==Additional Information==<br />
<br />
Put your text here<br />
<br />
==Links==<br />
<br />
None<br />
<br />
==References==<br />
<references /></div>Bailey.Glen