Savanah D. Gisriel, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA
Ji Yuan, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Ryan C. Braunberger, Department of Pathology, University of New Mexico, Albuquerque, NM, USA
Danielle L V Maracaja, Department of Pathology, Wake Forest School of Medicine, Winston-Salem, NC, USA
Xueyan Chen, Department of Laboratory Medicine and Pathology, University of Washington Medical Center, Seattle, WA, USA
Xiaojun Wu, Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD, USA
Jenna McCracken, Department of Pathology, Duke University Medical Center, Durham, NC, USA
Mingyi Chen, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA
Yi Xie, Department of Laboratory Medicine, University of California San Francisco, San Francisco, CA, USA
Laura E. Brown, Department of Laboratory Medicine, University of California San Francisco, San Francisco, CA, USA
Peng Li, ARUP Laboratories, University of Utah School of Medicine, Salt Lake City, UT, USA
Yi Zhou, Department of Pathology, University of Miami Health Systems, Miami, FL, USA
Tarsheen Sethi, Department of Medicine (Hematology), Yale University School of Medicine, New Haven, CT, USA
Austin McHenry, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA
Ronald G. Hauser, Department of Laboratory Medicine, Yale University School of Medicine, New Haven, CT, USA
Nathan Paulson, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA,Department of Pathology, Stanford University School of Medicine, Palo Alto, CA, USA
Haiming Tang, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA
Eric D. Hsi, Department of Pathology, Wake Forest School of Medicine, Winston-Salem, NC, USA
Endi Wang, Department of Pathology, Duke University Medical Center, Durham, NC, USA
Qian-Yun Zhang, Department of Pathology, University of New Mexico, Albuquerque, NM, USA
Ken H. Young, Department of Pathology, Duke University Medical Center, Durham, NC, USA
Mina L. Xu, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA
Zenggang Pan, Department of Pathology, Yale University School of Medicine, New Haven, CT, USA. zenggang.pan@cuanschutz.edu,Department of Pathology, University of Colorado School of Medicine, Aurora, CO, USA. zenggang.pan@cuanschutz.edu

Document Type

Article

Publication Date

10-1-2022

Abstract

Rare cases of human herpesvirus 8 (HHV8)-negative effusion-based large B-cell lymphoma (EB-LBCL) occur in body cavities without antecedent or concurrent solid mass formation. In contrast to HHV8 + primary effusion lymphoma (PEL), EB-LBCL has no known association with HIV or HHV8 infection. However, the small sample sizes of case reports and series worldwide, especially from non-Japanese regions, have precluded diagnostic uniformity. Therefore, we conducted a retrospective, multi-institutional study of 55 cases of EB-LBCL and performed a comprehensive review of an additional 147 cases from the literature to identify distinct clinicopathologic characteristics. In our study, EB-LBCL primarily affected elderly (median age 80 years), immunocompetent patients and manifested as lymphomatous effusion without a solid component. The lymphomatous effusions mostly occurred in the pleural cavity (40/55, 73%), followed by the pericardial cavity (17/55, 31%). EB-LBCL expressed CD20 (53/54, 98%) and PAX5 (23/23, 100%). Most cases (30/36, 83%) were of non-germinal center B-cell subtype per the Hans algorithm. HHV8 infection was absent (0/55, 0%), while Epstein-Barr virus was detected in 6% (3/47). Clinically, some patients were managed with drainage alone (15/34, 44%), while others received rituximab alone (4/34, 12%) or chemotherapy (15/34, 44%). Eventually, 56% (22/39) died with a median overall survival (OS) of 14.9 months. Our findings were similar to those from the literature; however, compared to the non-Japanese cases, the Japanese cases had a significantly higher incidence of pericardial involvement, a higher rate of chemotherapy administration, and longer median OS. Particularly, we have found that Japanese residence, presence of pericardial effusion, and absence of MYC rearrangement are all favorable prognostic factors. Our data suggest that EB-LBCL portends a worse prognosis than previously reported, although select patients may be managed conservatively. Overall, EB-LBCL has distinct clinicopathologic characteristics, necessitating the establishment of separate diagnostic criteria and consensus nomenclature.

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