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Updated: Aug 10, 2026

Identifying Dysregulated Genes Induced by Kaposi's Sarcoma-associated Herpesvirus (KSHV)
Published on: September 14, 2010
KSHV- and EBV-associated germinotropic lymphoproliferative disorder
Ming-Qing Du1, Tim C Diss, Hongxiang Liu
1Department of Histopathology, Royal Free and University College Medical School, University College London, Rockefeller Building, University Street, London WC1E 6JJ, UK. m.du@ucl.ac.uk
Abstract:
Kaposi sarcoma-associated herpesvirus (KSHV) is known to be associated with 3 distinct lymphoproliferative disorders: primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma. We report 3 cases of a previously undescribed KSHV-associated lymphoproliferative disorder. The disease presented as localized lymphadenopathy and showed a favorable response to chemotherapy or radiotherapy. Histologically, the lymphoproliferation is characterized by plasmablasts that preferentially involved germinal centers of the lymphoid follicles, forming confluent aggregates. They were negative for CD20, CD27, CD79a, CD138, BCL6, and CD10 but showed monotypic kappa or lambda light chain. Clusters of CD10(+)CD20(+) residual follicle center cells were identified in some of the follicles. The plasmablasts were positive for both KSHV and EBV, and most of them also expressed viral interleukin-6 (vIL-6). Unexpectedly, molecular analysis of whole tissue sections or microdissected KSHV-positive aggregates demonstrated a polyclonal or oligoclonal pattern of immunoglobulin (Ig) gene rearrangement. The plasmablasts showed somatic mutation and intraclonal variation in the rearranged Ig genes, and one case expressed switched Ig heavy chain (IgA), suggesting that they originated from germinal center B cells. We propose calling this distinctive entity "KSHV-associated germinotropic lymphoproliferative disorder."
Insights
We identified a new lymphoproliferative disorder associated with Kaposi sarcoma-associated herpesvirus (KSHV). This disorder, termed KSHV-associated germinotropic lymphoproliferative disorder, responds well to treatment.
Area of Science:
- Oncology
- Virology
- Pathology
Background:
- Kaposi sarcoma-associated herpesvirus (KSHV) is linked to primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma.
- A novel KSHV-associated lymphoproliferative disorder has been identified.
Purpose of the Study:
- To describe the clinical, histological, and molecular features of a previously undescribed KSHV-associated lymphoproliferative disorder.
- To propose a name for this new entity: KSHV-associated germinotropic lymphoproliferative disorder.
Main Methods:
- Case series analysis of three patients with the novel disorder.
- Histopathological examination including immunohistochemistry (CD20, CD27, CD79a, CD138, BCL6, CD10, kappa/lambda light chains).
- Molecular analysis of immunoglobulin (Ig) gene rearrangement, KSHV, EBV, and viral interleukin-6 (vIL-6) expression.
Main Results:
- The disorder presented as localized lymphadenopathy with a favorable response to chemotherapy or radiotherapy.
- Histology revealed plasmablasts in germinal centers, negative for B-cell markers but positive for KSHV and EBV, often expressing vIL-6.
- Molecular analysis showed polyclonal or oligoclonal Ig gene rearrangement with somatic mutation, suggesting germinal center B-cell origin.
Conclusions:
- A distinct KSHV-associated lymphoproliferative disorder, characterized by germinotropic plasmablasts, has been identified.
- The findings suggest an origin from germinal center B cells with evidence of somatic hypermutation.
- This entity, named KSHV-associated germinotropic lymphoproliferative disorder, warrants recognition and further study.
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