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Published on: November 8, 2011
Fibrin-associated EBV-positive Large B-Cell Lymphoma: An Indolent Neoplasm With Features Distinct From Diffuse Large
Daniel F Boyer1, Penelope A McKelvie, Laurence de Leval
1*Department of Pathology, University of Michigan School of Medicine, Ann Arbor, MI †Department of Pathology, The James Homer Wright Pathology Laboratories of the Massachusetts General Hospital, Harvard Medical School, Boston ‡‡Department of Pathology, North Shore Medical Center, Salem, MA ‡Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Vic., Australia §Institut Universitaire de Pathologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland ∥Department of Laboratory Medicine, University of Washington, Seattle, WA ¶Department of Pathology, Samsung Medical Center, Sungkyunkwan University, Seoul, Republic of Korea #Herbert Wertheim College of Medicine, Florida International University, Miami, FL **Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN ††Department of Pathology, Holmes Regional Medical Center, Melbourne, FL §§NeoGenomics Laboratories, Aliso Viejo, CA ∥∥Department of Pathology, Johns Hopkins University School of Medicine, Baltimore ¶¶Laboratory of Pathology, Hematopathology Section, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Fibrin-associated Epstein-Barr virus (EBV)+ large B-cell proliferations are distinct from diffuse large B-cell lymphoma with chronic inflammation (DLBCL-CI). These EBV+ proliferations, often found incidentally, may represent a distinct entity with a generally indolent course, potentially curable by surgery.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Localized Epstein-Barr virus (EBV)+ large B-cell proliferations are incidentally found at unusual sites.
- These have been categorized under diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) in the WHO Classification.
Purpose of the Study:
- To define the clinicopathologic spectrum of fibrin-associated EBV+ large B-cell proliferations.
- To compare their features with typical DLBCL-CI cases.
Main Methods:
- Analysis of 12 cases of fibrin-associated EBV+ large B-cell proliferations.
- Literature review of similar cases.
- Comparison with traditional DLBCL-CI cases.
Main Results:
- The 12 cases were incidental findings in atrial myxomas, thrombi, hematomas, and pseudocysts.
- All cases were nongerminal center B-cell origin with type III EBV latency, negative for MYC rearrangements and ALT by FISH.
- Most cases showed high CD30, Ki67, PD-L1, and low to moderate MYC and p53 expression.
Conclusions:
- Fibrin-associated EBV+ large B-cell lymphoma is clinicopathologically distinct from DLBCL-CI.
- Cases associated with pseudocysts behave indolently, potentially curable by surgery alone.
- Cardiac or vascular lesions may have a higher recurrence risk despite chemotherapy.
