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
PubMed

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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