Comparison of KSHV-associated lymphoproliferative disorders
| . | PEL . | MCD and associated plasmablastic lymphoma . | GLD . |
|---|---|---|---|
| Clinical presentation | In immunodeficient patients, with systemic symptoms, poor prognosis | Predominantly in immunodeficient patients, with systemic symptoms, poor prognosis | In immunocompetent patients with localized lymphadenopathy, favorable response to therapy |
| Sites | Body cavities, extranodal sites | Lymph nodes, spleen | Lymph nodes |
| Morphology | Immunoblasts with pleomorphic nuclei and abundant plasmacytoid cytoplasm | Plasmablasts, preferentially residing in the mantle zone | Plasmablasts, preferentially invading germinal centers |
| EBV | Present in HIV-associated patients; absent in HIV-negative patients | Negative | Positive |
| Cytoplasmic Ig expression | Absent | High, always IgM | High, any heavy chain |
| Ig light chain | Monotypic κ or λ mRNA | Monotypic λ | Monotypic κ or λ |
| CD30 | Positive | Weakly positive | Variable |
| B-cell antigens | Absent | Weak or absent | Absent |
| Mutation in Ig genes | Mutated in most | Absent | Mutated |
| Cellular origin | Germinal center or postgerminal center B cells | Naive IgMλ expressing B cells | Germinal center B cells |
| . | PEL . | MCD and associated plasmablastic lymphoma . | GLD . |
|---|---|---|---|
| Clinical presentation | In immunodeficient patients, with systemic symptoms, poor prognosis | Predominantly in immunodeficient patients, with systemic symptoms, poor prognosis | In immunocompetent patients with localized lymphadenopathy, favorable response to therapy |
| Sites | Body cavities, extranodal sites | Lymph nodes, spleen | Lymph nodes |
| Morphology | Immunoblasts with pleomorphic nuclei and abundant plasmacytoid cytoplasm | Plasmablasts, preferentially residing in the mantle zone | Plasmablasts, preferentially invading germinal centers |
| EBV | Present in HIV-associated patients; absent in HIV-negative patients | Negative | Positive |
| Cytoplasmic Ig expression | Absent | High, always IgM | High, any heavy chain |
| Ig light chain | Monotypic κ or λ mRNA | Monotypic λ | Monotypic κ or λ |
| CD30 | Positive | Weakly positive | Variable |
| B-cell antigens | Absent | Weak or absent | Absent |
| Mutation in Ig genes | Mutated in most | Absent | Mutated |
| Cellular origin | Germinal center or postgerminal center B cells | Naive IgMλ expressing B cells | Germinal center B cells |