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HHV8-Positive Germinotropic Lymphoproliferative Disorder

MedGen UID:
1656332
•Concept ID:
C4725706
•
Neoplastic Process
Synonyms: Germinotropic lymphoproliferative disorder caused by Human herpesvirus 8; HHV8-positive germinotropic lymphoproliferative disorder
SNOMED CT: Germinotropic lymphoproliferative disorder caused by Human herpesvirus 8 (787125004); HHV8-positive germinotropic lymphoproliferative disorder (787125004)

Definition

A monotypic HHV8-positive lymphoproliferative disorder that usually occurs in HIV-negative individuals. It is characterized by the presence of HHV8-positive plasmablasts partially or completely replacing germinal centers. Coinfection with EBV is characteristic. In most cases, there is a favorable response to chemotherapy or radiation. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHHV8-Positive Germinotropic Lymphoproliferative Disorder

Recent clinical studies

Etiology

KSHV/HHV8-positive large B-cell lymphomas and associated diseases: a heterogeneous group of lymphoproliferative processes with significant clinicopathological overlap.
Vega F, Miranda RN, Medeiros LJ
Mod Pathol 2020 Jan;33(1):18-28. Epub 2019 Sep 16 doi: 10.1038/s41379-019-0365-y. PMID: 31527708

Diagnosis

Molecular Features of HHV8 Monoclonal Microlymphoma Associated with Kaposi Sarcoma and Multicentric Castleman Disease in an HIV-Negative Patient.
Rogges E, Pelliccia S, Savio C, Lopez G, Della Starza I, La Verde G, Di Napoli A
Int J Mol Sci 2024 Mar 28;25(7) doi: 10.3390/ijms25073775. PMID: 38612584Free PMC Article
Cavity-based lymphomas: challenges and novel concepts. A report of the 2022 EA4HP/SH lymphoma workshop.
Di Napoli A, Soma L, Quintanilla-Martinez L, de Leval L, Leoncini L, Zamò A, Ng SB, Ondrejka SL, Climent F, Wotherspoon A, Dirnhofer S
Virchows Arch 2023 Sep;483(3):299-316. Epub 2023 Aug 9 doi: 10.1007/s00428-023-03599-2. PMID: 37555981Free PMC Article
Human herpesvirus 8-positive multicentric Castleman disease with germinotropic plasmablastic aggregates: Overlapping spectrum of human herpesvirus 8-associated lymphoproliferative disorder.
Nakaya Y, Ishii N, Kasamatsu Y, Shimizu K, Tatsumi N, Tsutsumi M, Yoshida M, Yoshimura T, Hayashi Y, Nakao T, Inoue T, Yamane T
Pathol Int 2020 Aug;70(8):574-580. Epub 2020 May 24 doi: 10.1111/pin.12951. PMID: 32449234
KSHV/HHV8-positive large B-cell lymphomas and associated diseases: a heterogeneous group of lymphoproliferative processes with significant clinicopathological overlap.
Vega F, Miranda RN, Medeiros LJ
Mod Pathol 2020 Jan;33(1):18-28. Epub 2019 Sep 16 doi: 10.1038/s41379-019-0365-y. PMID: 31527708
HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma.
Gonzalez-Farre B, Martinez D, Lopez-Guerra M, Xipell M, Monclus E, Rovira J, Garcia F, Lopez-Guillermo A, Colomo L, Campo E, Martinez A
Mod Pathol 2017 May;30(5):745-760. Epub 2017 Jan 13 doi: 10.1038/modpathol.2016.233. PMID: 28084335

Prognosis

Cavity-based lymphomas: challenges and novel concepts. A report of the 2022 EA4HP/SH lymphoma workshop.
Di Napoli A, Soma L, Quintanilla-Martinez L, de Leval L, Leoncini L, Zamò A, Ng SB, Ondrejka SL, Climent F, Wotherspoon A, Dirnhofer S
Virchows Arch 2023 Sep;483(3):299-316. Epub 2023 Aug 9 doi: 10.1007/s00428-023-03599-2. PMID: 37555981Free PMC Article
Lymphoproliferative disorders with concurrent HHV8 and EBV infection: beyond primary effusion lymphoma and germinotropic lymphoproliferative disorder.
Wang W, Kanagal-Shamanna R, Medeiros LJ
Histopathology 2018 Apr;72(5):855-861. Epub 2018 Jan 3 doi: 10.1111/his.13428. PMID: 29105119
HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma.
Gonzalez-Farre B, Martinez D, Lopez-Guerra M, Xipell M, Monclus E, Rovira J, Garcia F, Lopez-Guillermo A, Colomo L, Campo E, Martinez A
Mod Pathol 2017 May;30(5):745-760. Epub 2017 Jan 13 doi: 10.1038/modpathol.2016.233. PMID: 28084335

Clinical prediction guides

HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma.
Gonzalez-Farre B, Martinez D, Lopez-Guerra M, Xipell M, Monclus E, Rovira J, Garcia F, Lopez-Guillermo A, Colomo L, Campo E, Martinez A
Mod Pathol 2017 May;30(5):745-760. Epub 2017 Jan 13 doi: 10.1038/modpathol.2016.233. PMID: 28084335

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