Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
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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.
The term "plasmablastic" is used for this lymphoma because the cells resemble plasma cells and have abundant immunoglobulin, but they lack IG somatic hyper-mutation.
This lymphoma is considered to be the same primary with the following:
1. Plasmablastic lymphoma (9735/3). The two diagnosis can be distinguished because plasmablastic lymphoma frequently show class-switched and hyper-mutated IG genes.
2. 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.