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Code grade specified by pathologist. If no grade specified, code 9
This is a neoplasm with diffuse proliferation of large neoplastic cells, most of which resemble B immunoblasts, but in which all tumor cells have the immunophenotype of plasma cells. This disease is uncommon. The highest percentage occurs in patients who are HIV positive.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9684/3.) PBL is uncommon. The highest incidence is in HIV-positive individuals. Usually presents as a mass in the oral cavity but also occurs in other extranodal sites particularly mucosal sites. Other primary sites include the sinonasal cavity, orbit, skin, bone, soft tissues and GI tract. Nodal involvement is uncommon. Most patients present at Stages III/IV. CT and PET scans may show disseminated bone involvement. The international prognostic index (IPI) is of intermediate to high risk. Patients who are HIV negative present more commonly in lymph nodes. EBV positivity is seen in the majority of patients.
Definitive Diagnostic Methods
Clonal IgH light chain rearrangement
CD20 weakly positive
CD45 weakly positive
CD56- in oral mucosal primary
Cytoplasmic immunoglobulins expressed, most frequently IgG and either kappa or lambda light chain