Kaposi sarcoma herpesvirus/human herpesvirus‐8–negative effusion‐based lymphoma: Report of 3 cases and review of the literature

Jian, Xi Gao; Selvaggi, Suzanne M.; Leith, Catherine P.; Fitzgerald, Sean A.; Stewart, Jimmie

doi:10.1002/cncy.21311

Cited by 19 publications

(22 citation statements)

References 44 publications

(56 reference statements)

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“…Morphologically, HHV8-negative EBL typically consist of predominantly large cells with irregular nuclear contours, coarse chromatin, multiple conspicuous nucleoli that are often pleomorphic, and abundant vacuolated cytoplasm. 16 IHC usually confirms B-cell origin in HHV8-negative EBL. In one case series, 86.7% of HHV8-negative EBL cases demonstrated pan-B-cell markers whereas the incidence of pan-B-cell markers in PEL has been reported at 39.8%.…”

Section: Discussionmentioning

confidence: 80%

“…Unlike PEL, whose morphology is either large cell or small Burkitt‐like, morphology in HHV8‐negative EBL is predominantly large cell type with B‐cell phenotype. Morphologically, HHV8‐negative EBL typically consist of predominantly large cells with irregular nuclear contours, coarse chromatin, multiple conspicuous nucleoli that are often pleomorphic, and abundant vacuolated cytoplasm 16 . IHC usually confirms B‐cell origin in HHV8‐negative EBL.…”

Section: Discussionmentioning

confidence: 96%

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Primary age‐related EBV‐associated effusion‐based lymphoma successfully treated with rituximab and thoracentesis

Kuhlman

Moustafa

Tun

et al. 2021

Clinical Case Reports

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We report a 98-year-old patient with primary age-related EBV-associated effusion-based lymphoma (aEBV-EBL) in the setting of persistent transudative pleural effusion. He was treated with rituximab and thoracentesis achieving complete remission. We suggest that aEBV-EBL is a subtype of EBVpositive HHV8-negative EBL, which is part of the EBVpositive DLBCL-NOS spectrum.Primary human herpesvirus 8-negative effusion-based lymphoma (HHV8-negative EBL) is a distinct category of non-Hodgkin lymphoma which manifests as a serous effusion without a distinct tumor mass. The hallmark of a HHV8-negative EBL diagnosis is negativity for HHV8 infection, thereby distinguishing itself from primary effusion lymphoma (PEL) which requires the presence of HHV8 infection. 1 HHV8-negative EBL has been found in association with hepatitis C virus (HCV) infection, fluid overload, liver cirrhosis, renal dysfunction, cardiac arrhythmias, myocardial infarction, and heart failure. [2][3][4][5] Epstein-Barr virus (EBV) infection has been reported in 13% of HHV8-negative EBL cases. 3 EBV-positive and HHV8-negative EBL have been reported in patients with liver disease, HIV, lymphocytopenia, common variable immunodeficiency, solid organ transplant, and renal disease. 2,[6][7][8][9][10] EBV-positive DLBCL-NOS is a well-defined entity in the World Health Organization (WHO) classification of B-cell lymphomas. EBV-positive and HHV8-negative EBL can be defined as EBV-positive DLBCL-NOS primarily presenting as EBL. Age-related EBV-associated DLBCL, another wellknown entity seen in elderly patients, is also recognized as part of the spectrum of EBV-positive DLBCL-NOS. 11,12 This entity is known to follow an aggressive clinical course when compared against EBV-negative DLBCL. 13,14 Herein, we report an extremely rare case of primary age-related EBV-associated effusion-based lymphoma in a 98-year-old man who achieved complete remission following rituximab monotherapy and thoracentesis. We further discuss

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 96%

Primary age‐related EBV‐associated effusion‐based lymphoma successfully treated with rituximab and thoracentesis

Kuhlman

Moustafa

Tun

et al. 2021

Clinical Case Reports

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“…These occur in HIV-negative and older individuals than KSHV-associated PEL, representing a different disease entity (73–78). …”

Section: Text Of Reviewmentioning

confidence: 99%

How do viruses trick B cells into becoming lymphomas?

Cesarman

2014

Current Opinion in Hematology

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Purpose of review Since the discovery of EBV in Burkitt lymphoma 50 years ago, only one other virus, namely KSHV/HHV-8, has been confirmed to be a direct cause of B cell lymphoma. Here we will review the evidence for EBV and KSHV as causal lymphoma agents. Recent findings A deeper understanding of specific mechanisms by which EBV and KSHV cause B cell lymphomas has been acquired over the past years, in particular with respect to viral protein interactions with host cell pathways, microRNA functions. Specific therapies based on knowledge of viral functions are beginning to be evaluated, mostly in pre-clinical models. Summary Understanding the causal associations of specific infections agents with certain B cell lymphomas has allowed more accurate diagnosis and classification. A deeper knowledge of the specific mechanisms of transformation is essential to begin assessing whether virus-targeted treatment modalities may be used in the future.

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“…3 Immunophenotypically, the neoplastic cells usually express plasmacytic but not B-cell markers. [1][2][3]5,6 The distinction from PEL is important, as EBL shows a relatively better prognosis and an occasional spontaneous regression after effusion drainage. 1,4 Effusion-based lymphoma (EBL) is an even rarer entity and is unrelated to HHV-8.…”

mentioning

confidence: 99%

“…1,4 Effusion-based lymphoma (EBL) is an even rarer entity and is unrelated to HHV-8. [1][2][3]5,6 The distinction from PEL is important, as EBL shows a relatively better prognosis and an occasional spontaneous regression after effusion drainage. 2,3,7,8 EBL patients are typically older than PEL patients, and generally HIV-negative.…”

mentioning

confidence: 99%

Effusion‐based lymphoma with morphological regression but with clonal genetic features after aspiration

Tsai

Kuo

et al. 2018

Diagnostic Cytopathology

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Effusion-based lymphoma (EBL) is a rare but distinct entity of large B-cell lymphoma in effusion without association with human herpes virus-8 (HHV-8). Spontaneous regression after pleurocentesis has been observed; but to our knowledge, there are no reports on the morphological and molecular features of subsequent aspirations in regressing cases. Here, we report the case of a 92-year-old male with chronic obstructive pulmonary disease, who presented with right pleural effusion. He had no human immunodeficiency virus, hepatitis B virus, or hepatitis C virus infection, and CT scans revealed no mass lesion. The first pleural effusion aspiration cytology revealed large lymphoma cells with vesicular nuclei, irregular nuclear contours, and prominent nucleoli, consistent with EBL. The second aspiration cytology showed a few slightly enlarged lymphocytes in a background of small lymphocytes. Immunohistochemical study on cell block of the second aspiration revealed equal amounts of CD3-positive and CD20-positive cells. All these cells on the section tested negative for HHV-8 through immunohistochemistry and Epstein-Barr virus by in situ hybridization. Our initial impression was EBL in regression. However, flow cytometric immunophenotyping showed monotypic light chain expression of the gated B-cells. B-cell receptor gene rearrangement study showed a clonal result. Furthermore, fluorescence in situ hybridization revealed rearrangement of IGH gene. The diagnosis of the second aspiration was EBL with morphological regression but retained clonal genetic features. The patient passed away one month after diagnosis without chemotherapy. This case illustrated the importance of ancillary studies in confirming the clonal nature of a morphologically regressing EBL.

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Kaposi sarcoma herpesvirus/human herpesvirus‐8–negative effusion‐based lymphoma: Report of 3 cases and review of the literature

Cited by 19 publications

References 44 publications

Primary age‐related EBV‐associated effusion‐based lymphoma successfully treated with rituximab and thoracentesis

Primary age‐related EBV‐associated effusion‐based lymphoma successfully treated with rituximab and thoracentesis

How do viruses trick B cells into becoming lymphomas?

Effusion‐based lymphoma with morphological regression but with clonal genetic features after aspiration

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