HAEM4:Multicentric Castleman Disease: Difference between revisions

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==Primary Author(s)*==

Multicentric Castleman Disease

None

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.

Angio follicular lymph-node hyperplasia  

Giant node hyperplasia  

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.

==References==

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