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WHO Classification of Tumours
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
Tumours of haematopoietic and lymphoid tissues


Definition

Large B-cell lymphoma arising in human herpes virus 8 (HHV8)-associated multicentric Castleman disease (HHV8 MCD) is composed of a monoclonal proliferation of HHV8-infected lymphoid cells resembling plasmablasts expressing IgMλ and arising in the setting of multicentric Castleman disease (MCD). It is usually associated with human immunodeficiency virus (HIV) infection. The term plasmablastic is used for this lymphoma because the cells morphologically resemble plasma cells and have abundant cytoplasmic immunoglobulin; however it corresponds to a na´ve, IgM-producing plasma cell without IG somatic hypermutation. This lymphoma must be distinguished from plasmablastic lymphomas presenting in the oral cavity or other extranodal sites that frequently show class-switched and hypermutated IG genes.

> Associated conditions
Kaposi sarcoma is frequently present in patients with HHV8-positive plasmablastic lymphoma (HHV8 PL) and MCD. Primary effusion lymphoma (PEL) and its extracavitary counterpart may complicate HHV8 MCD but unlike HHV8 PL, the neoplastic cells do not express Ig and are usually co-infected with EBV.

In the germinotropic lymphoproliferative disorder, another monotypic HHV8-positive lymphoproliferative lesion that occurs in HIV negative individuals, HHV8-positive plasmablasts infiltrate germinal centres

Click to access Pubmed
Du MQ, Diss TC, Liu H, Ye H, Hamoudi RA, Cabešadas J, Dong HY, Harris NL, Chan JK, Rees JW, Dogan A, Isaacson PG (2002)
KSHV- and EBV-associated germinotropic lymphoproliferative disorder.
Blood 100: 3415-8



. The plasmablasts show either κ or λ light chain restriction but, like HHV8 MCD, these cells are polyclonal. Co-infection with EBV is also characteristic.

> Related Topics
Plasmablastic lymphoma
Primary effusion lymphoma
Introduction: Classification of lymphoid neoplasms