Lymphoproliferative disorders with concurrent <scp>HHV</scp>8 and <scp>EBV</scp> infection: beyond primary effusion lymphoma and germinotropic lymphoproliferative disorder

Wang, Wei; Kanagal‐Shamanna, Rashmi; Medeiros, L. Jeffrey

doi:10.1111/his.13428

Cited by 21 publications

(17 citation statements)

References 27 publications

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“…However, rare cases of HHV8+ DLBCL also show evidence of EBV infection. 116 Molecular HHV8+ DLBCL carries clonal immunoglobulin gene rearrangements without evidence of somatic hypermutation of the variable region genes. 1 MYC rearrangement has been reported, but the frequency is unknown.…”

Section: Immunophenotypementioning

confidence: 99%

Diffuse large B-cell lymphoma variants: an update

et al. 2020

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Section: Immunophenotypementioning

confidence: 99%

Diffuse large B-cell lymphoma variants: an update

et al. 2020

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“…These HHV-8-associated LPDs constitute a spectrum of disorders which can be distinguished from one another based on their clinical manifestations, microscopic appearance, localization, immunophenotype, genetic profile, and the presence of an Epstein–Barr virus (EBV) coinfection. However, there have been reported cases that have overlapping features of several of these HHV-8-associated LPDs, and which may also manifest clinically in ways distinct from these WHO-classified disorders [5–7].…”

Section: Introductionmentioning

confidence: 99%

HHV-8-Associated Lymphoproliferative Disorders and Pathogenesis in an HIV-Positive Patient

Guerrero

Jain

Kelemen

2019

Case Reports in Hematology

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Human herpesvirus 8 (HHV-8), also known as Kaposi’s sarcoma-associated herpesvirus, is a DNA oncovirus known for its role in the development of Kaposi’s sarcoma (KS) and several lymphoproliferative disorders (LPDs). HHV-8 promotes lymphoproliferation via the activation of the interleukin-6 receptor signaling pathway, as well as a host of other regulatory mechanisms. The spectrum of HHV-8-associated LPDs is increasing. The World Health Organization has recently updated the classification of HHV-8-associated LPDs by introducing HHV-8-positive germinotropic LPD (GLPD) in addition to the previously recognized entities of HHV-8-positive diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), primary effusion lymphoma (PEL), and HHV-8-positive multicentric Castleman’s disease (MCD). We present here a case of an HIV-positive woman with a history of KS, who later developed three HHV-8-associated LPDs, including HHV-8-positive MCD, PEL, and GLPD. To the best of our knowledge, this is the first reported case of a patient with this combination of individually rare HHV-8-associated LPDs. This case illustrates the spectrum and the sequential development of the different clinical manifestations of HHV-8-associated diseases. Detection of HHV-8 can have clinical significance in the diagnosis and management of certain HHV-8-associated conditions. Recently discovered variants of HHV-8-associated LPDs indicate that this group represents a diverse spectrum of disorders, whose classification may require further refinement beyond the currently recognized entities.

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“…PEL is a non‐Hodgkin lymphoma occurring in immunodeficiency settings, mainly in HIV‐infected individuals, usually presenting as a malignant serosal effusion without lymphadenopathy. Association with Epstein‐Barr virus is found in approximately 80%‐90% of cases . KS lesions are found in one third of patients diagnosed with PEL .…”

Section: Discussionmentioning

confidence: 99%

The spectrum of the cytopathological features of primary effusion lymphoma and human herpes virus 8‐related lymphoproliferative disorders

et al. 2020

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Introduction: Human herpes virus 8-related lymphoproliferative disorders are a complex and heterogeneous group of entities and some of them are eminently diagnosed by cytopathology. In a routine laboratory, these lesions account for less than 1% of the effusion fluids samples. However, they represent up to 30% of all the lymphoma diagnosis from effusion cytological samples and their consideration in the diagnostic flow chart is mandatory, especially in human immunodeficiency virus-positive patients. Methods: A retrospective series of cytological specimens from cavity effusions (n = 605) were analysed. Five human herpes virus 8-related lymphoproliferative processes were recruited. A combination of morphological criteria (enhanced with May-Grünwald Giemsa staining), cell block-based immunocytochemistry and flow cytometry were undertaken for final characterisation. Results: The identification of malignant cells may be difficult. Some specimens are particularly rich, easily leading to suspect a lymphoproliferative process, whereas in other cases, the presence of abundant reactive mesothelial cells, histiocytes, neutrophils, small reactive T and B lymphocytes may obscure the neoplastic process. The biological behaviour may be very heterogeneous and a standardised therapy for these cases is still lacking, although some patients may benefit from antiretroviral therapy in a human immunodeficiency virus setting. Conclusions: The present case series highlights some characteristic findings of these entities to reaffirm useful cytopathological diagnostic criteria, stressing the crucial role of the appropriate technical processing of effusion fluids to obtain the best performances.

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Lymphoproliferative disorders with concurrent HHV8 and EBV infection: beyond primary effusion lymphoma and germinotropic lymphoproliferative disorder

Abstract: The description of these two cases suggests that the category of HHV8 EBV LPDs is a spectrum, which not only includes PEL and germinotropic LPD, but also other cases that have overlapping but distinctive features.

Cited by 21 publications

References 27 publications

Diffuse large B-cell lymphoma variants: an update

Diffuse large B-cell lymphoma variants: an update

HHV-8-Associated Lymphoproliferative Disorders and Pathogenesis in an HIV-Positive Patient

The spectrum of the cytopathological features of primary effusion lymphoma and human herpes virus 8‐related lymphoproliferative disorders

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