HHV8-negative effusion based lymphoma: a series of 17 cases at a single institution

Usmani, Amena; Walts, Ann E.; Patel, Snehalkumar; Alkan, Serhan; Kitahara, Sumire

doi:10.1016/j.jasc.2014.10.002

Cited by 11 publications

(21 citation statements)

References 29 publications

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“…The majority of HHV8 negative effusion lymphomas occur in HIV-negative elderly patients. [ 12 ] Failure of these tumor cells to stain with keratin or melanoma markers and immunoreactivity for HHV8 helps differentiate PEL from metastasis.…”

Section: Discussionmentioning

confidence: 99%

Human Immunodeficiency Virus-associated primary effusion lymphoma: An exceedingly rare entity in cerebrospinal fluid

et al. 2015

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Primary effusion lymphoma (PEL) in patients with Human Immunodeficiency Virus (HIV) infection may involve pleural, pericardial, and peritoneal cavities. PEL involving the cerebrospinal fluid (CSF) is exceedingly rare, and to our knowledge has only been reported in two cases. We report another case of PEL diagnosed in CSF from a 61-year-old male with Acquired Immunodeficiency Syndrome that presented with neurological symptoms. Imaging studies of his brain showed leptomeningeal/periventricular enhancement, but no mass lesion. His CSF demonstrated human herpesvirus-8 positive pleomorphic lymphoplasmacytoid cells of null cell phenotype. This case highlights that albeit rare, PEL should be included in the differential diagnosis when large atypical cells are encountered in CSF of HIV-positive patients, even when such patients have no history of lymphoma. As in this case, ancillary studies are required to make an accurate diagnosis of PEL in CSF cytology.

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Section: Discussionmentioning

confidence: 99%

Human Immunodeficiency Virus-associated primary effusion lymphoma: An exceedingly rare entity in cerebrospinal fluid

et al. 2015

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“…Unlike in PEL, in which the tumor cells usually lack pan B-cell markers, the majority of tumor cells in HHV8-negative effusion-based indolent lymphoma express pan B-cell markers and do not express an activation marker or plasma cell-associated antigen (15). The tumor cells in the present case also expressed pan B-cell markers, although not all cells demonstrated uniform and strong expression of pan B-cell marker; no tumor cells expressed activation markers or plasma cell-associated antigens except MUM1.…”

Section: Discussionmentioning

confidence: 90%

Human herpesvirus 8‑negative effusion‑based lymphoma with indolent clinical behavior in an elderly patient: A case report and literature review

Kim

Yoon

et al. 2020

Oncol Lett

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Primary effusion lymphoma (PEL) is a B-cell non-Hodgkin's lymphoma that is usually characterized by lymphomatous effusions in the body cavity without any detectable tumor masses. According to the World Health Organization (WHO) schema for tumor classification, PEL is defined by the presence of human herpesvirus 8 (HHV8) in malignant lymphoid cells. However, a subset of effusion-based B-cell lymphoma is not HHV8-positive and exhibits different clinicopathological characteristics. The 2017 WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues does not list HHV8-negative effusion-based lymphoma, which remains an underappreciated B-cell lymphoma, as an individual entity. The present study reports a case of this rare type of lymphoma with indolent clinical behavior in a 75-year-old male patient receiving only symptomatic treatment. Additionally, a review of similar cases reported in the English literature is presented.

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“…In the revised 2017 World Health Organization (WHO) classification of lymphoid neoplasms, PEL is defined as a large B‐cell neoplasm universally associated with the human herpesvirus 8 (HHV8), occurring most often in the setting of immunodeficiency, most frequently in HIV infected patients . In contrast, HHV8‐negative (HHV8−) effusion‐based lymphoma (EBL) has been proposed, while consensus criteria for diagnosis have not yet been achieved . HHV8‐negative EBL is under‐recognized, and constitutes a clinicopathological entity with distinct clinical, immunophenotypic, and prognostic features that distinguish it from PEL .…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, HHV8‐negative (HHV8−) effusion‐based lymphoma (EBL) has been proposed, while consensus criteria for diagnosis have not yet been achieved . HHV8‐negative EBL is under‐recognized, and constitutes a clinicopathological entity with distinct clinical, immunophenotypic, and prognostic features that distinguish it from PEL . In our recent study of PEL in Taiwan with a literature review of 256 cases, we identified a group of patients with HHV8‐negative EBL and with rare HIV infection, in contrast to the HIV‐ and HHV8‐double positive classical PEL in the WHO classification .…”

Section: Discussionmentioning

confidence: 99%

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Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Liu

Chen

Chuang

2020

Diagnostic Cytopathology

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Background Extranodal natural killer/T‐cell lymphoma (ENKTL) nasal type is an extranodal lymphoma, with most cases arising from the prototypic nasal/upper aerodigestive tract and the cell of origin (COO) is frequently of NK cells. Effusion involvement by ENKTL is rare. The cytomorphological and immunophenotypical features and COO of effusion ENKTL are elusive. Design We report four new cases of effusion ENKTL, reviewing the cytomorphological features and investigating their COO by immunohistochemistry for T‐cell receptor (TCR) protein expression and clonality assay for TCR gene rearrangement. We also reviewed 12 additional cases of effusion ENKTL from the literature. Results All our four cases showed anaplastic tumor cells with fine to coarse azurophilic granules, with the primary tumor organs from the prototypic sites (2), small intestine (1), or indeterminate (1). The COO was either T‐cell (3) or NK‐cell (1). Compiling our four cases with that from the literature, all 16 effusion ENKTL cases were Epstein‐Barr virus‐associated and the majority (83%; 10/12) originated from the nonprototypic sites. Five of six (83%) effusion ENKTL cases were of T‐cell lineage. Prognosis was poor with a median survival time of only 2.4 months. Conclusions Our study revealed that ENKTL in the effusion might be characterized by anaplastic large cell morphology, cytoplasmic azurophilic granules, and a higher proportion of T‐cell lineage as compared to de novo tumors arising from the prototypic sites. ENKTL rarely presented in effusion, which is associated with dismal outcome. Large international studies are warranted to validate these findings.

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HHV8-negative effusion based lymphoma: a series of 17 cases at a single institution

Cited by 11 publications

References 29 publications

Human Immunodeficiency Virus-associated primary effusion lymphoma: An exceedingly rare entity in cerebrospinal fluid

Human Immunodeficiency Virus-associated primary effusion lymphoma: An exceedingly rare entity in cerebrospinal fluid

Human herpesvirus 8‑negative effusion‑based lymphoma with indolent clinical behavior in an elderly patient: A case report and literature review

Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

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