Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics

Gisriel, Savanah D.; Braunberger, Ryan C; Maracaja, Danielle L V; Chen, Xueyan; Wu, Xiaojun; McCracken, Jenna; Chen, Mingyi; Xie, Youhua; Brown, Laura; Zhou, Yi; Sethi, Tarsheen; McHenry, Austin; Hauser, Ronald G.; Paulson, Nathan; Tang, Haiming; Hsi, Eric D.; Wang, Endi; Zhang, Qianyun; Young, Ken H.; Xu, Mina L.; Pan, Zenggang

doi:10.1038/s41379-022-01091-x

Cited by 16 publications

(18 citation statements)

References 70 publications

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“…Thus, the differential diagnosis of a pleural effusion-associated lymphoma includes not only PEL but also fluid overload-associated large B-cell lymphoma (FO-LBCL, KSHV/HHV8negative effusion-based lymphoma) as well as diffuse large B-cell lymphoma associated with chronic inflammation (CI-DLBCL, pyothorax-associated lymphoma), particularly among rare cases of TKI-associated pleural effusion. Table 1 compares PEL, EBL, and PAL in terms of patient characteristics, clinical presentation, etiology, and immunophenotype [1,27,28,29,30,31].…”

Section: Discussionmentioning

confidence: 99%

Primary Effusion Lymphoma in an HIV-Negative Patient with Chronic Myeloid Leukemia Treated with Dasatinib

SahBandar¹,

Sy²,

Akker³

et al. 2023

Pathobiology

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Introduction: Primary effusion lymphoma (PEL) is a malignant lymphomatous effusion, which by definition is Kaposi sarcoma herpesvirus/human herpesvirus 8-positive. PEL typically occurs in HIV-infected patients, but can also occur in HIV-negative individuals, including in organ transplant recipients. Tyrosine kinase inhibitors (TKIs) are currently the standard of care for patients with chronic myeloid leukemia (CML), BCR::ABL1-positive. Although TKIs are extremely effective in treating CML, they alter T-cell function by inhibiting peripheral T-cell migration and altering T-cell trafficking and have been associated with the development of pleural effusions. Case Presentation: We report a case of PEL in a young, relatively immunocompetent patient with no history of organ transplant receiving dasatinib for CML, BCR::ABL1-positive. Discussion: We hypothesize that the loss of T-cell function secondary to TKI therapy (dasatinib) may have resulted in the unchecked cellular proliferation of KSHV-infected cells, leading to the emergence of a PEL. We recommend cytologic investigation and KSHV testing in patients being treated with dasatinib for CML who present with persistent or recurrent effusions.

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Section: Discussionmentioning

confidence: 99%

Primary Effusion Lymphoma in an HIV-Negative Patient with Chronic Myeloid Leukemia Treated with Dasatinib

SahBandar¹,

Sy²,

Akker³

et al. 2023

Pathobiology

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“…Hu et al 16 in their series of 70 PEL included 2 tumors affecting the skin/buccal region and Mate et al 12 described a solid PEL affecting the tongue of a 42-year-old HIV-positive male patient who died 2 weeks after treatment initiation. In 1997 Buske et al 64 Other lymphomas manifesting as cavitary effusions must be distinguished from classic PEL, 66 while the list of differential diagnoses for the solid variant is also complex. Lymphomas characterized by a plasmablastic differentiation represent the most important entities to be distinguished from extracavitary PEL (Table 2).…”

Section: Discussionmentioning

confidence: 99%

“…Other lymphomas manifesting as cavitary effusions must be distinguished from classic PEL, 66 while the list of differential diagnoses for the solid variant is also complex. Lymphomas characterized by a plasmablastic differentiation represent the most important entities to be distinguished from extracavitary PEL (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Extracavitary Primary Effusion Lymphoma Affecting the Oral Cavity: A Rare Case Report

et al. 2023

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Primary effusion lymphoma (PEL) is an aggressive neoplasm often diagnosed in immunosuppressed patients demonstrating peritoneal, pleural, or pericardial effusions. This high-grade lymphoma is strongly associated with human herpesvirus 8 (HHV8) infection and most of the lesions also show the presence of Epstein–Barr virus in tumor cells, which lacks CD20 expression and reveals a plasmablastic morphology and phenotype. The extracavitary or solid variant of PEL is even rarer and usually affects the lymph nodes and is currently considered a clinical manifestation of the classic PEL. In the oral cavity, extracavitary PEL is extremely rare and only a few patients have been previously reported, with no detailed clinicopathological description. The recognition of oral extracavitary PEL is even more important given the occurrence of plasmablastic lymphoma in the oral mucosa, which shares many clinical, microscopic, and phenotypic features with PEL, therefore, demanding from pathologists the search for HHV8, especially in immunosuppressed patients, and an appropriate clinical evaluation. In this report, we aim to describe a very rare extracavitary PEL affecting the palate of a 36-year-old patient and to review the literature regarding the extracavitary presentation of this aggressive lymphoma. This report demonstrates the importance of searching for HHV8 infection in oral lymphomas with plasmablastic features.

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“…Although HHV8‐unrelated PEL‐like lymphoma (PEL‐LL) is morphologically indistinguishable from PEL, they present with distinctive cell markers. PEL‐LL is universally positive for pan‐B cell markers (CD19, CD20, and CD79a), commonly expressing BCL‐2 and MUM1, rarely positive for plasma cell differentiation markers (CD138), and negative for CD10 and light chain restriction, whereas PEL is negative for pan‐B cell markers (CD19, CD20, CD79a) but usually positive for CD45, CD30, CD38, CD71, epithelial membrane antigen, CD138, VS38c, and MUM‐1/IRF4 2,3 . Recent studies found PEL‐LL to be associated with underlying medical conditions (e.g., liver cirrhosis) and fluid overload 4 .…”

Section: Introductionmentioning

confidence: 99%

“…PEL‐LL is universally positive for pan‐B cell markers (CD19, CD20, and CD79a), commonly expressing BCL‐2 and MUM1, rarely positive for plasma cell differentiation markers (CD138), and negative for CD10 and light chain restriction, whereas PEL is negative for pan‐B cell markers (CD19, CD20, CD79a) but usually positive for CD45, CD30, CD38, CD71, epithelial membrane antigen, CD138, VS38c, and MUM‐1/IRF4. 2 , 3 Recent studies found PEL‐LL to be associated with underlying medical conditions (e.g., liver cirrhosis) and fluid overload. 4 In clinical practice, despite the relatively indolent nature of PEL‐LL, the high prevalence of underlying medical conditions such as HBV‐related liver cirrhosis still significantly challenged treatment planning.…”

Section: Introductionmentioning

confidence: 99%

HHV8‐unrelated primary effusion lymphoma in a patient with HBV‐related liver cirrhosis: A case successfully treated with rituximab and lenalidomide

Liao

Yuan

Wei

2023

Clinical Case Reports

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BACKGROUNDPrimary effusion lymphoma (PEL) is a rare subtype of non-Hodgkin lymphoma (NHL), characterized by malignant pleural, pericardial, or peritoneal primary effusions without distinguishable extra-cavitary tumor masses and is found tightly associated with human herpesvirus type 8/Kaposi sarcoma-associated herpes virus (HHV8/KSHV) and sometimes coinfected with Epstein-Barr virus (EBV). 1 Although HHV8-unrelated PEL-like lymphoma (PEL-LL) is morphologically indistinguishable from PEL, they present with distinctive cell markers. PEL-LL is universally positive for pan-B cell markers (CD19, CD20, and CD79a), commonly expressing BCL-2 and MUM1, rarely positive for plasma cell differentiation markers (CD138), and negative for CD10 and light chain restriction, whereas PEL is negative for pan-B cell markers (CD19, CD20, CD79a) but usually positive for CD45, CD30, CD38, CD71, epithelial membrane antigen, CD138, VS38c, and MUM-1/IRF4. 2,3 Recent studies found PEL-LL to be associated with underlying medical conditions (e.g., liver cirrhosis) and fluid overload. 4 In clinical practice, despite the relatively indolent nature of PEL-LL, the high prevalence of underlying medical conditions such as HBV-related liver cirrhosis

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Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics

Cited by 16 publications

References 70 publications

Primary Effusion Lymphoma in an HIV-Negative Patient with Chronic Myeloid Leukemia Treated with Dasatinib

Primary Effusion Lymphoma in an HIV-Negative Patient with Chronic Myeloid Leukemia Treated with Dasatinib

Extracavitary Primary Effusion Lymphoma Affecting the Oral Cavity: A Rare Case Report

HHV8‐unrelated primary effusion lymphoma in a patient with HBV‐related liver cirrhosis: A case successfully treated with rituximab and lenalidomide

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