Table 3

Differential diagnosis of PBL

PBLPlasmablastic myelomaLBCL HHV-8–positiveIBL DLBLCALK-positive DLBCLDLBCL ACIPEL
Disease distribution Extranodal Bone marrow and extranodal Nodal Nodal Nodal Extranodal Extranodal 
HIV infection ∼70% No Yes No No No Yes 
Pathogenesis EBV, HIV, IL-10 IL-6 HHV-8, MCD  ALK EBV, IL-10, IL-6 HHV-8 
Positive markers CD138, IRF-4/MUM-1, MYC CD138, cytoplasmic Ig, MYC CD20−/+, CD138+/−, IgM CD20, PAX-5, ALK, CD4, CD45 CD20, CD4 IRF-4/MUM-1, CD30−/+ 
Negative markers CD20, PAX-5 CD20, PAX-5, BCL-6 CD138 CD4, CD138 CD20, CD30, MYC ALK PAX-5, CD20, CD138, Ig 
Proliferation rate >90% >90% >90% ∼80% >90% >90% >90% 
Cytoplasmic immunoglobulin 50%-70% >90% IgA λ Uncommon Uncommon Uncommon Uncommon 
EBV infection Common No No No No Common Common 
EBV latency pattern NA NA NA NA III 
HHV-8 infection No No Yes No No No Yes 
Molecular genetics MYC GR Myeloma FISH abnormalities Unmutated Ig MYC GR t(2;17)(p23;q23) TP53 mutations Hypermutated Is 
PBLPlasmablastic myelomaLBCL HHV-8–positiveIBL DLBLCALK-positive DLBCLDLBCL ACIPEL
Disease distribution Extranodal Bone marrow and extranodal Nodal Nodal Nodal Extranodal Extranodal 
HIV infection ∼70% No Yes No No No Yes 
Pathogenesis EBV, HIV, IL-10 IL-6 HHV-8, MCD  ALK EBV, IL-10, IL-6 HHV-8 
Positive markers CD138, IRF-4/MUM-1, MYC CD138, cytoplasmic Ig, MYC CD20−/+, CD138+/−, IgM CD20, PAX-5, ALK, CD4, CD45 CD20, CD4 IRF-4/MUM-1, CD30−/+ 
Negative markers CD20, PAX-5 CD20, PAX-5, BCL-6 CD138 CD4, CD138 CD20, CD30, MYC ALK PAX-5, CD20, CD138, Ig 
Proliferation rate >90% >90% >90% ∼80% >90% >90% >90% 
Cytoplasmic immunoglobulin 50%-70% >90% IgA λ Uncommon Uncommon Uncommon Uncommon 
EBV infection Common No No No No Common Common 
EBV latency pattern NA NA NA NA III 
HHV-8 infection No No Yes No No No Yes 
Molecular genetics MYC GR Myeloma FISH abnormalities Unmutated Ig MYC GR t(2;17)(p23;q23) TP53 mutations Hypermutated Is 

ACI, associated with chronic inflammation; ALK, anaplastic lymphoma kinase; FISH, fluorescence in situ hybridization; GR, gene rearrangement; IBL, immunoblastic; IL, interleukin; IRF-4/MUM-1, interferon regulatory factor 4/multiple myeloma 1; LBCL, large B-cell lymphoma; NA, not available; PEL, primary effusion lymphoma.

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