HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome

Bacha, Dhouha; Chelly, Beya; Kilani, Houda; Charfi, Lamia; Douggaz, Amel; Chatti, Samia; Chelbi, Emna

doi:10.1155/2017/1578429

Cited by 5 publications

(6 citation statements)

References 12 publications

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“…La presencia de células atípicas en las áreas interfoliculares y de la zona del manto plantea la posibilidad de migración de linfocitos B neoplásicos a los centros germinales por ecotaxis/reubicación (35). El análisis molecular de plasmablastos positivos para KSHV en GLPD ha demostrado un patrón policlonal u oligoclonal de reordenamiento del gen de la inmunoglobulina, lo que lleva a la terminología propuesta de "trastorno linfoproliferativo germinotrópico asociado con KSHV y EBV (GLPD)" (31) . El inmunofenotipo convencionalmente descrito es la positividad de las células tumorales para HHV8, cadenas ligeras kappa o lambda, MUM1 y Ki67 en la mayoría de las células.…”

Section: Discussionunclassified

“…Esta entidad presenta un amplio rango de cambios histológicos, desde plasmablastos ubicados en la zona del manto hasta microlinfomas monoclonales que pueden derivar en linfomas plasmablásticos; los plasmablastos solo presentan infección por HHV8/KSVH, expresan IgMƛ y también tienen niveles altos de Ig citoplasmática con CD27 de superficie, similar a las células B maduras, siendo su origen en las células B vírgenes al no presentar hipermutacón somática en el gen de las inmunoglobulinas. Las dos entidades antes presentadas llevan un curso clínico agresivo, diferente a lo que sucede con el DLG (30,31) Linfoma de Células Grandes B Difuso positivo para HHV8 NOS generalmente surge en asociación con la enfermedad multicéntrica de Castleman y la infección por VIH (32) . Se encontraron algunos casos en ausencia de enfermedad multicéntrica de Castleman.…”

Section: • Metástasis De Carcioma Linfoepitelial Delunclassified

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Desorden linfoproliferativo germinotrópico asociado al HHV8/KSVH y EBV

Schwarz¹,

Villena

Poma

et al. 2022

Interciencia méd.

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Describimos el caso de un paciente portador de Desorden Linfoproliferativo Germinotrópico (DLG) Asociado a Virus Epstein Baar (EBV) y herpesvirus 8 (HHV8/KSVH), el primer reporte de esta entidad se realizo en la revista Blood del año 2002 (1) y a la fecha se han descrito alrededor de 20 casos (2,3); esta clasificada dentro de los desordenes linfoproliferativos asociados al herpesvirus 8 (HHV8/KSVH) (4)(5). Clínicamente se caracteriza por presentarse en pacientes inmunocompetentes, como una adenopatía localizada, de curso indolente, con respuesta favorable a la quimioterapia, radioterapia o escisión quirúrgica (6). Histológicamente se observan plasmablastos que marcan positivo a EBV y HHV8/KSVH formando agregados confluentes que tienden a invadir los centros germinales (germinotropismo) (6); al hacer la microdisección de los plasmablastos y estudio cadena de reacción de polimesasa (PCR), muestran un patrón policlonal u oligoclonal, por esto es catalogado como un “desorden” y no como una neoplasia propiamente dicha (6). La linfomagenesis esta dada por la co-infección de una célula B de origen centrogerminal; inicialmente tenemos a las células B como reservorio latente del HHV8/KSVH y posteriormente recibe el “second genetic hit” por el Virus Epstein Barr (EBV) (7), desarrollando en un paciente inmunocompetente el DLG.

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Section: Discussionunclassified

Section: • Metástasis De Carcioma Linfoepitelial Delunclassified

Desorden linfoproliferativo germinotrópico asociado al HHV8/KSVH y EBV

Schwarz¹,

Villena

Poma

et al. 2022

Interciencia méd.

View full text Add to dashboard Cite

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“…9 Such cases may well represent particular diagnostic challenges, particularly regarding the distinction between MCD with plasmablastic aggregates (previously termed 'microlymphoma'), GLPD, and EC-PEL. Here, we report two cases of LPD associated with HHV8 and EBV that further expand the spectrum of HHV8/EBV-positive lymphoproliferations [29][30][31][32][33][34][35][36][37][38][39] (Table S2). Thus, HHV8/ EBV-positive LPDs probably represent a broad spectrum of lesions with overlapping features and possible evolution among different entities [29][30][31][32][33][34][35][36][37][38][39] (Table S3).…”

Section: Discussionmentioning

confidence: 99%

“…Here, we report two cases of LPD associated with HHV8 and EBV that further expand the spectrum of HHV8/EBV-positive lymphoproliferations [29][30][31][32][33][34][35][36][37][38][39] (Table S2). Thus, HHV8/ EBV-positive LPDs probably represent a broad spectrum of lesions with overlapping features and possible evolution among different entities [29][30][31][32][33][34][35][36][37][38][39] (Table S3).…”

Section: Discussionmentioning

confidence: 99%

Epstein–Barr virus reactivation influences clonal evolution in human herpesvirus‐8‐related lymphoproliferative disorders

et al. 2021

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Background Human herpesvirus‐8 (HHV8) is a lymphotropic virus associated with different lymphoproliferative disorders, including primary effusion lymphoma (PEL), multicentric Castleman’s disease (MCD), diffuse large B‐cell lymphomas, not otherwise specified, and the rare entity known as germinotropic lymphoproliferative disorder (GLPD). In PELs and GLPD the neoplastic cells also contain Epstein–Barr virus (EBV). In addition, occasional cases with atypical and overlapping features among these entities have been recognised, suggesting that the spectrum of the HHV8‐related lesions may not be fully characterised. Aims Here, we report two cases of lymphoproliferative disorder associated with HHV8 and EBV that further expand the spectrum of HHV8/EBV‐positive lymphoproliferative disease. Methods and results Case 1 represented HHV8/EBV‐positive extracavitary nodal PEL followed by pleural PEL. The striking characteristic of this case was the almost focal and intrasinusoidal localisation of the neoplastic cells and the association with Castleman’s disease features. In the second case, we found the entire spectrum of HHV8‐related disorders, i.e. MCD, GLPD, and PEL, coexisting in the same lymph node, underlining the variability, possible overlap and evolution among these entities. Both cases were well analysed with immunohistochemistry, determination of the EBV latency programme, and molecular analysis for clonality of immnoglobulin genes. In both patients, the disease followed an unexpected indolent course, both being still alive after 8 and 12 months, respectively. Conclusion Our findings represent further evidence of the overlap among HHV8/EBV‐positive lymphoproliferative disorders, and underline a grey zone that requires further study; they further confirm the experimental evidence that lytic EBV replication influences HHV8‐related tumorigenesis.

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“…1 Currently, after 17 years of its first descripcion, 2 only 18 cases of this very infrequent disease has been published. [2][3][4][5][6][7][8][9][10][11][12][13] The histological pattern of GLPD comprises monotypic plasmablasts that involve the germinal centers of the lymphoid follicles (germinotrophism), forming confluent aggregates positive for both human herpes virus type 8 (HHV8) and Epstein-Barr virus (EBV). Unlike other HHV8-associated lymphoproliferative disorders, namely, primary effusion lymphoma (PEL), multicentric Castleman's disease (MCD), and HHV8-positive diffuse large B-cell lymphoma (HHV8-DLBCL), GLPD mainly affects immunocompetent (HIVnegative) individuals.…”

Section: Introductionmentioning

confidence: 99%

Intrasinusoidal HHV8-EBV–Positive Large B-Cell Lymphoma With Features of Germinotropic Lymphoproliferative Disorder

et al. 2020

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Germinotropic lymphoproliferative disorder (GLPD) is a poorly characterized lymphoproliferative entity, recently included in the World Health Organization classification of hematolymphoid neoplasms. The histological pattern of this disease comprises monotypic plasmablasts that involve the germinal centers of the lymphoid follicles (germinotrophism), forming confluent aggregates positive for both human herpes virus type 8 (HHV8) and Epstein-Barr virus. Currently, after 17 years of its first description, only 18 cases have been reported. In this article, we describe a case of a GLPD presenting in an immunocompetent 79-year-old woman with localized axillary lymphadenopathy, showing a prominent sinusoidal growth pattern, with no evidence of germinotrophism or extrasinusoidal spread. Stinking pleomorphism in tumor cells was also noted. An extension study has not revealed involvement of other groups of lymph nodes or extralymphoid sites. The patient is alive and has shown no relapse after 8 years follow-up (the longest follow-up reported period for this entity). This previously unrecognized pure sinusoidal growth pattern along with the striking pleomorphism in neoplastic cells closely mimics the appearance of an anaplastic large cell lymphoma. GLPD is not usually considered in such a setting, but it should be included in the differential diagnosis of sinusoidal proliferations. Our findings contribute to the expansion of the morphological spectrum of HHV8-associated lymphoproliferative lesions and aids in the characterization of the very infrequent GLPD entity.

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HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome

Cited by 5 publications

References 12 publications

Desorden linfoproliferativo germinotrópico asociado al HHV8/KSVH y EBV

Desorden linfoproliferativo germinotrópico asociado al HHV8/KSVH y EBV

Epstein–Barr virus reactivation influences clonal evolution in human herpesvirus‐8‐related lymphoproliferative disorders

Intrasinusoidal HHV8-EBV–Positive Large B-Cell Lymphoma With Features of Germinotropic Lymphoproliferative Disorder

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