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

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

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Introduction: Human herpesvirus 8-positive (HHV8+) primary effusion lymphoma is a well-recognized clinicopathologic entity. In contrast, HHV8-negative (HHV8-) effusion-based lymphoma (EBL) is incompletely characterized and under-recognized. We describe 17 cases of HHV8- EBL at our institution. Materials and methods: Cytology and available immunohistochemistry and cytogenetics were reviewed. Patient demographics, history, and outcome were obtained from medical records. Results: The effusions were pleural (n = 9; 53%), peritoneal (n = 4; 24%), pericardial (n = 3; 18%), and pleural and pericardial (n = 1; 6%). Fifteen cases (88%) were CD20+ and 15 had sufficient information for classification by Hans algorithm (CD10, BCL6, MUM1): 11 (73%) nongerminal center and 4 (27%) germinal center phenotype. Epstein-Barr virus in situ hybridization was negative in 16 cases (94%). Three of 14 cases were MYC+ by immunohistochemistry. Fluorescence in situ hybridization, performed on 5 of the 17 cases, showed a MYC rearrangement in 1 case and a BCL6 rearrangement in 2 cases. Most patients were elderly (median age 86 years) and female (82%). Human immunodeficiency virus testing results, available in 4 patients, were negative. Seven (41%), including 1 of 2 heart transplant recipients, had congestive heart failure. Follow-up (5 days to 12 years) was available for 16 patients including 4 who survived ≥8 years. Only 1 of the 8 known deaths was clinically attributed to lymphoma. Conclusions: HHV8- lymphomas that occur in body cavity effusions without detectable lymphomatous masses are usually composed of large CD20+ lymphoid or lymphoplasmacytoid cells. In contrast to HHV8+ primary effusion lymphoma, patients with HHV8- EBL are usually elderly, lack a documented human immunodeficiency virus-positive history, and have a longer disease specific survival.

Original languageEnglish (US)
Pages (from-to)37-43
Number of pages7
JournalJournal of the American Society of Cytopathology
Volume4
Issue number1
DOIs
StatePublished - Jan 1 2015
Externally publishedYes

Fingerprint

Lymphoma
Primary Effusion Lymphoma
History
Immunohistochemistry
HIV
Human Herpesvirus 8
Germinal Center
Pleural Effusion
Fluorescence In Situ Hybridization
Human Herpesvirus 4
Cytogenetics
Medical Records
In Situ Hybridization
Cell Biology
Heart Failure
Demography
Phenotype
Survival

Keywords

  • Effusion lymphoma
  • HHV8-negative effusion-based lymphoma
  • Human herpesvirus 8
  • Primary effusion lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

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.

In: Journal of the American Society of Cytopathology, Vol. 4, No. 1, 01.01.2015, p. 37-43.

Research output: Contribution to journalArticle

Usmani, Amena ; Walts, Ann E. ; Patel, Snehalkumar ; Alkan, Serhan ; Kitahara, Sumire. / HHV8-negative effusion based lymphoma : A series of 17 cases at a single institution. In: Journal of the American Society of Cytopathology. 2015 ; Vol. 4, No. 1. pp. 37-43.
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abstract = "Introduction: Human herpesvirus 8-positive (HHV8+) primary effusion lymphoma is a well-recognized clinicopathologic entity. In contrast, HHV8-negative (HHV8-) effusion-based lymphoma (EBL) is incompletely characterized and under-recognized. We describe 17 cases of HHV8- EBL at our institution. Materials and methods: Cytology and available immunohistochemistry and cytogenetics were reviewed. Patient demographics, history, and outcome were obtained from medical records. Results: The effusions were pleural (n = 9; 53{\%}), peritoneal (n = 4; 24{\%}), pericardial (n = 3; 18{\%}), and pleural and pericardial (n = 1; 6{\%}). Fifteen cases (88{\%}) were CD20+ and 15 had sufficient information for classification by Hans algorithm (CD10, BCL6, MUM1): 11 (73{\%}) nongerminal center and 4 (27{\%}) germinal center phenotype. Epstein-Barr virus in situ hybridization was negative in 16 cases (94{\%}). Three of 14 cases were MYC+ by immunohistochemistry. Fluorescence in situ hybridization, performed on 5 of the 17 cases, showed a MYC rearrangement in 1 case and a BCL6 rearrangement in 2 cases. Most patients were elderly (median age 86 years) and female (82{\%}). Human immunodeficiency virus testing results, available in 4 patients, were negative. Seven (41{\%}), including 1 of 2 heart transplant recipients, had congestive heart failure. Follow-up (5 days to 12 years) was available for 16 patients including 4 who survived ≥8 years. Only 1 of the 8 known deaths was clinically attributed to lymphoma. Conclusions: HHV8- lymphomas that occur in body cavity effusions without detectable lymphomatous masses are usually composed of large CD20+ lymphoid or lymphoplasmacytoid cells. In contrast to HHV8+ primary effusion lymphoma, patients with HHV8- EBL are usually elderly, lack a documented human immunodeficiency virus-positive history, and have a longer disease specific survival.",
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AU - Walts, Ann E.

AU - Patel, Snehalkumar

AU - Alkan, Serhan

AU - Kitahara, Sumire

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N2 - Introduction: Human herpesvirus 8-positive (HHV8+) primary effusion lymphoma is a well-recognized clinicopathologic entity. In contrast, HHV8-negative (HHV8-) effusion-based lymphoma (EBL) is incompletely characterized and under-recognized. We describe 17 cases of HHV8- EBL at our institution. Materials and methods: Cytology and available immunohistochemistry and cytogenetics were reviewed. Patient demographics, history, and outcome were obtained from medical records. Results: The effusions were pleural (n = 9; 53%), peritoneal (n = 4; 24%), pericardial (n = 3; 18%), and pleural and pericardial (n = 1; 6%). Fifteen cases (88%) were CD20+ and 15 had sufficient information for classification by Hans algorithm (CD10, BCL6, MUM1): 11 (73%) nongerminal center and 4 (27%) germinal center phenotype. Epstein-Barr virus in situ hybridization was negative in 16 cases (94%). Three of 14 cases were MYC+ by immunohistochemistry. Fluorescence in situ hybridization, performed on 5 of the 17 cases, showed a MYC rearrangement in 1 case and a BCL6 rearrangement in 2 cases. Most patients were elderly (median age 86 years) and female (82%). Human immunodeficiency virus testing results, available in 4 patients, were negative. Seven (41%), including 1 of 2 heart transplant recipients, had congestive heart failure. Follow-up (5 days to 12 years) was available for 16 patients including 4 who survived ≥8 years. Only 1 of the 8 known deaths was clinically attributed to lymphoma. Conclusions: HHV8- lymphomas that occur in body cavity effusions without detectable lymphomatous masses are usually composed of large CD20+ lymphoid or lymphoplasmacytoid cells. In contrast to HHV8+ primary effusion lymphoma, patients with HHV8- EBL are usually elderly, lack a documented human immunodeficiency virus-positive history, and have a longer disease specific survival.

AB - Introduction: Human herpesvirus 8-positive (HHV8+) primary effusion lymphoma is a well-recognized clinicopathologic entity. In contrast, HHV8-negative (HHV8-) effusion-based lymphoma (EBL) is incompletely characterized and under-recognized. We describe 17 cases of HHV8- EBL at our institution. Materials and methods: Cytology and available immunohistochemistry and cytogenetics were reviewed. Patient demographics, history, and outcome were obtained from medical records. Results: The effusions were pleural (n = 9; 53%), peritoneal (n = 4; 24%), pericardial (n = 3; 18%), and pleural and pericardial (n = 1; 6%). Fifteen cases (88%) were CD20+ and 15 had sufficient information for classification by Hans algorithm (CD10, BCL6, MUM1): 11 (73%) nongerminal center and 4 (27%) germinal center phenotype. Epstein-Barr virus in situ hybridization was negative in 16 cases (94%). Three of 14 cases were MYC+ by immunohistochemistry. Fluorescence in situ hybridization, performed on 5 of the 17 cases, showed a MYC rearrangement in 1 case and a BCL6 rearrangement in 2 cases. Most patients were elderly (median age 86 years) and female (82%). Human immunodeficiency virus testing results, available in 4 patients, were negative. Seven (41%), including 1 of 2 heart transplant recipients, had congestive heart failure. Follow-up (5 days to 12 years) was available for 16 patients including 4 who survived ≥8 years. Only 1 of the 8 known deaths was clinically attributed to lymphoma. Conclusions: HHV8- lymphomas that occur in body cavity effusions without detectable lymphomatous masses are usually composed of large CD20+ lymphoid or lymphoplasmacytoid cells. In contrast to HHV8+ primary effusion lymphoma, patients with HHV8- EBL are usually elderly, lack a documented human immunodeficiency virus-positive history, and have a longer disease specific survival.

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KW - HHV8-negative effusion-based lymphoma

KW - Human herpesvirus 8

KW - Primary effusion lymphoma

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