Primary mediastinal (thymic) large B cell lymphoma with aberrant expression of CD3: a case report with review of the literature.

Journal Article (Journal Article;Review)

We report the first case of primary mediastinal large B cell lymphoma (PMBL) with aberrant expression of CD3. PMBL is a subtype of diffuse large B cell lymphoma (DLBCL) and usually presents with bulky mediastinal lesions. Lineage ambiguity/infidelity is uncommon in DLBCL but has been described in sporadic case reports/series. A literature search identifies 13 additional cases of DLBCL expressing CD3, with the majority displaying cytoplasmic expression. Of the 14 total cases, 6 are pyothorax-associated lymphoma, 4 are conventional DLBCL, 2 are plasmablastic lymphoma, one is primary effusion lymphoma and one is PMBL. Two cases show genotypic ambiguity/infidelity with dual clonal IG and TCR gene rearrangements in addition to ambiguous immunophenotypes. Of the 13 cases tested for EBV status, 11 are positive, suggesting an important role of EBV in promoting lineage ambiguity/infidelity. A low threshold for testing EBV status is advocated in DLBCL with phenotypic ambiguity along with panels of immunohistochemical and molecular studies.

Full Text

Duke Authors

Cited Authors

  • Wang, E; Stoecker, M

Published Date

  • April 2010

Published In

Volume / Issue

  • 91 / 3

Start / End Page

  • 509 - 515

PubMed ID

  • 20131102

Electronic International Standard Serial Number (EISSN)

  • 1865-3774

Digital Object Identifier (DOI)

  • 10.1007/s12185-010-0501-4


  • eng

Conference Location

  • Japan