Lymphoma & related disorders
HHV8 associated lymphoproliferative disorders
HHV8 related germinotropic lymphoproliferative disorder


Minor changes: 5 April 2021

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PubMed Search: HHV8 germinotropic lymphoproliferative disorder

Julie Teruya-Feldstein, M.D.
Page views in 2021 to date: 37
Cite this page: Venkateswaran J, Teruya-Feldstein J. HHV8 related germinotropic lymphoproliferative disorder. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaHHV8germinotropicLPD.html. Accessed April 18th, 2021.
Definition / general
  • Monotypic HHV8 positive lymphoproliferative lesion occurring characteristically in HIV negative patients
Essential features
  • Coinfection of HHV8 and EBV
  • Immunocompetent patients
  • Localized lymphadenopathy
  • Aggregates or single plasmablasts / immunoblasts in the germinal centers
  • Good prognosis
Epidemiology
  • No known epidemiological association
  • Usually seen in HIV negative, immunocompetent patients
  • Occasional cases seen in HIV positive patients
Sites
  • Involves lymph nodes
Pathophysiology
  • Uncertain contribution of EBV in pathogenesis
  • Occasional EBV negative cases
  • Elusive role of vIL6
    • Activation of IL6 receptor signaling through vIL6 and human IL6 may play a role in differentiation of Kaposi sarcoma associated herpesvirus infected B cells into plasmablasts and development of lymphoproliferative lesions (Am J Surg Pathol 2017;41:795)
Etiology
  • Unclear origin of the plasmablasts; possible theories include:
Clinical features
  • Localized, sometimes multifocal lymphadenopathy in otherwise healthy individuals
Diagnosis
  • Lymph node biopsy
  • Morphology and immunohistochemistry
  • Immunophenotype and molecular studies
Radiology description
  • Very few descriptions of radiological features reported so far
Prognostic factors
  • Overall favorable response to chemotherapy and radiation
  • Rare reported cases progressed to high grade HHV8+ EBV+ lymphoma
Case reports
Treatment
  • Treatment approach is variable; some cases have not been treated, while others have been treated with surgery alone or with chemotherapy and radiation
  • Potential therapeutic role of PD1 / PDL1 immunotherapy has been suggested due to the PDL1 expression and it requires additional research
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Julie Teruya-Feldstein, M.D.
Follicles of varying sizes

Follicles of varying sizes

Hyalinization in some follicles

Hyalinization in some follicles

Plasmablasts Plasmablasts

Plasmablasts


EBER1+

EBER1

Plasmablasts MUM1+

Plasmablasts positive for MUM1

Plasmablasts CD30-

Plasmablasts negative for CD30

Plasmablasts BCL6-

Plasmablasts negative for BCL6

Plasmablasts CD10-

Plasmablasts negative for CD10

Plasmablasts CD79a-

Plasmablasts negative for CD79a

Positive stains
Negative stains
Molecular / cytogenetics description
  • Plasmablasts are polyclonal or oligoclonal
Sample pathology report
  • Lymph node, excision / core needle biopsy:
    • HHV8 related germinotropic lymphoproliferative disorder
Differential diagnosis
Board review style question #1

Which of the following is true about HHV8 related germinotropic lymphoproliferative disorder?

  1. There is coinfection of EBV and HHV8
  2. Usually seen in HIV positive patients
  3. Positive for LMP1, EBNA2 and BZLF1 expression and negative for cytoplasmic monotypic immunoglobulin heavy chain
  4. Unfavorable response to chemotherapy and radiation
Board review style answer #1
A. There is coinfection of EBV and HHV8

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Reference: HHV8 related germinotropic lymphoproliferative disorder
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