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This disease is composed of HHV8-infected lymphoid cells resembling plasmablasts expressing IgM and arising in the setting of multicentric Castleman disease (MCD). It is usually associated with HIV.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9680/3.) The lymphoma characteristically involves lymph nodes and spleen but can disseminate to other viscera via the blood stream in which rarely it manifests as a leukemia. On a background of HHV8 CD, patients who are developing HHV8 PL present with profound immunodeficiency, enlarging lymph nodes and massive splenomegaly often accompanied by manifestations of Kaposi's Sarcoma.
The term "plasmablastic" is used for this lymphoma because the cells resemble plasma cells and have abundant immunoglobulin, but they lack IG somatic hypermutation. This lymphoma is considered to be the same primary as plasmablastic lymphoma (9735/3) but the two diagnosis can be distinguished because plasmablastic lymphoma frequently show class-switched and hypermutated IG genes. Primary effusion lymphoma (9678/3) may complicate HHV8 MCD but PEL neoplastic cells do no express immunoglobulin and are usually co-infected with EBV.
This is a very aggressive lymphoma with a median survival of a few months.
Note: Castleman disease by itself is not reportable.
Definitive Diagnostic Methods
IG genes are unmutated
CD20+ or CD20-
Epstein-Barr encoded RNA (EBER)-negative
Nuclear staining for interleukin-6
Nuclear staining for LANA-1
Strongly express cIgM with lambda light chain restriction