Fibrin-associated EBV-positive Large B-Cell Lymphoma: An Indolent Neoplasm with Features Distinct from Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation

Daniel F. Boyer, Penelope A. McKelvie, Laurence De Leval, Kerstin L. Edlefsen, Young Hyeh Ko, Zachary A. Aberman, Alexandra E. Kovach, Aneal Masih, Ha T. Nishino, Lawrence M. Weiss, Alan K. Meeker, Valentina Nardi, Maryknoll Palisoc, Lina Shao, Stefania Pittaluga, Judith A. Ferry, Nancy Lee Harris, Aliyah R. Sohani

Research output: Contribution to journalArticlepeer-review

Abstract

Incidental cases of localized fibrin-associated Epstein-Barr virus (EBV)+ large B-cell proliferations have been described at unusual anatomic sites and have been included in the category of diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) in the WHO Classification. We describe 12 cases and review the literature to define their clinicopathologic spectrum and compare features with typical cases of DLBCL-CI. Median age was 55.5 years with a M:F ratio of 3. In all 12 cases, the lymphoma was an incidental microscopic finding involving atrial myxomas (n=3), thrombi associated with endovascular grafts (n=3), chronic hematomas (n=2), and pseudocysts (n=4). All cases tested were nongerminal center B-cell origin, type III EBV latency, and were negative for MYC rearrangements and alternative lengthening of telomeres by FISH. Most showed high CD30, Ki67, and PD-L1, and low to moderate MYC and p53 expression. Among 11 patients with detailed follow-up, 6 were treated surgically, 3 with cardiac or vascular lesions had persistent/recurrent disease at intravascular sites, and 4 died of causes not directly attributable to lymphoma. Reports of previously published fibrin-associated cases showed similar features, whereas traditional DLBCL-CI cases with a mass lesion had significantly higher lymphoma-associated mortality. Fibrin-associated EBV+ large B-cell lymphoma is clinicopathologically distinct from DLBCL-CI, warranting separate classification. Most cases, particularly those associated with pseudocysts, behave indolently with the potential for cure by surgery alone and may represent a form of EBV+ lymphoproliferative disease rather than lymphoma. However, primary cardiac or vascular disease may have a higher risk of recurrence despite systemic chemotherapy.

Original languageEnglish (US)
Pages (from-to)299-312
Number of pages14
JournalAmerican Journal of Surgical Pathology
Volume41
Issue number3
DOIs
StatePublished - 2017

Keywords

  • Epstein-Barr virus
  • chronic inflammation
  • diffuse large B-cell lymphoma
  • fibrin
  • microscopic

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Fingerprint Dive into the research topics of 'Fibrin-associated EBV-positive Large B-Cell Lymphoma: An Indolent Neoplasm with Features Distinct from Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation'. Together they form a unique fingerprint.

Cite this