南区病理讨论会-高雄荣民总医院.pptVIP

Clinical Issues Oral?site: Most common site 90% of patients are HIV(+) 60% have localized disease (stage I) at diagnosis Involves gingiva, frequently bone (+) Non-oral site: Less frequently HIV(+) 60% are disseminated disease (stage IV) at diagnosis Maxillary sinus, nasopharynx, GI, and others * Pathogenesis/Etiology Not clearly defined Dysregulation of terminal B-cell differentiation and apoptosis, potentially via the effects of MYC translocation and EBV infection Immunodeficiency and EBV infection: Strongly associated with HIV infection Other including post-transplantation, autoimmune disease, and elderly * Pathology Diffuse growth pattern Frequent starry sky pattern with tingible body macrophages Apoptotic bodies and mitoses are usually numerous * Pathology Cytologic spectrum Immunoblastic Cells have prominent central nucleoli Plasmablastic Cells have more abundant cytoplasm and eccentrically located nuclei Binucleation or multinucleation is common * IHC Common pan-B-cell markers commonly absent CD20, CD22, and PAX5 CD79a is more often positive; also often weak intensity Strong positivity for plasma cell-associated markers MUM1(+), CD38(+), CD138 (+) Ki-67 is high: 70% in most cases * IHC CD30(+) in subset Aberrant expression of T-cell markers in some cases CD3, CD4, CD7, CD43 LMP 1 and 2 are not expressed EBV small encoded RNA (EBER) is positive in ~ 75% of cases Useful for distinguishing PBL from plasmablastic plasma cell myeloma? * IHC CD56 is variable in PBL, can be positive in cases with plasmablastic cytologic features. CD56 is commonly expressed in plasma cell myeloma MYC rearrangement in PBL Rare in plamacytoma * Clinical correlation is important Favor plamacytoma Presence of serum monoclonal protein (paraprotein) Excess light chains (Bence Jones protein) in urine Bone marrow involvement with radiologic evidence of lytic lesions EBER(-) Favor PBL Immunosuppression, especially HIV-related EBER(+) * Prognosis Poor prognosis, die within 1st