Related Experiment Videos
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
Blood
|October 18, 2002
Summary
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.