International Journal of Hematology

, Volume 91, Issue 3, pp 509–515

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

Case Report

Abstract

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.

Keywords

Large B cell lymphoma CD3 EBV 

References

  1. 1.
    Harris NL, Jaffe ES, Diebold J, et al. The World Health Organization classification of neoplasms of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997. Hematol J. 2000;1:53–66.CrossRefPubMedGoogle Scholar
  2. 2.
    Barth TF, Leithäuser F, Joos S, et al. Mediastinal (thymic) large B-cell lymphoma: where do we stand? Lancet Oncol. 2002;3(4):229–34.CrossRefPubMedGoogle Scholar
  3. 3.
    Tagawa H, Suguro M, Tsuzuki S, et al. Comparison of genome profiles for identification of distinct subgroups of diffuse large B-cell lymphoma. Blood. 2005;106:1770–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Mitrovic Z, Ilic I, Nola M, et al. CD43 expression is an adverse prognostic factor in diffuse large B-Cell lymphoma. Clin Lymphoma Myeloma. 2009;9:133–7.CrossRefPubMedGoogle Scholar
  5. 5.
    Wang J, Chen C, Lau S, et al. CD3-positive large B-cell lymphoma. Am J Surg Pathol. 2009;33:505–12.CrossRefPubMedGoogle Scholar
  6. 6.
    Wallentine JC, Perkins SL, Tripp SR, et al. Diffuse large B-cell lymphoma with coexpression of CD3 in a pediatric patient: a case report, review of the literature, and tissue microarray study. J Pediatr Hematol Oncol. 2009;31:124–7.CrossRefPubMedGoogle Scholar
  7. 7.
    Petitjean B, Jardin F, Joly B, et al. Pyothorax-associated lymphoma: a peculiar clinicopathologic entity derived from B cells at late stage of differentiation and with occasional aberrant dual B- and T-cell phenotype. Am J Surg Pathol. 2002;26:724–32.CrossRefPubMedGoogle Scholar
  8. 8.
    Al Saati T, Delécluze HJ, Chittal S. A novel human lymphoma cell line (Deglis) with dual B/T phenotype and gene rearrangements and containing Epstein–Barr virus genomes. Blood. 1992;80:209–16.PubMedGoogle Scholar
  9. 9.
    Mori N, Yatabe Y, Narita M, et al. Pyothorax-associated lymphoma. An unusual case with biphenotypic character of T and B cells. Am J Surg Pathol. 1996;20:760–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Beaty MW, Kumar S, Sorbara L, et al. A biphenotypic human herpesvirus 8-associated primary bowel lymphoma. Am J Surg Pathol. 1999;23:992–4.CrossRefPubMedGoogle Scholar
  11. 11.
    Hollingsworth HC, Stetler-Stevenson M, Gagneten D, et al. Immunodeficiency-associated malignant lymphoma. Three cases showing genotypic evidence of both T- and B-cell lineages. Am J Surg Pathol. 1994;18:1092–101.PubMedCrossRefGoogle Scholar
  12. 12.
    Cazals-Hatem D, Lepage E, Brice P. Primary mediastinal large B-cell lymphoma. A clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA (“Groupe d’Etude des Lymphomes de l’Adulte”) study. Am J Surg Pathol. 1996;20:877–88.CrossRefPubMedGoogle Scholar
  13. 13.
    Matutes E, Morilla R, Farahat N, et al. Definition of acute biphenotypic leukemia. Haematologica. 1997;82:64–6.PubMedGoogle Scholar
  14. 14.
    Ababas A, Lichtman AH, Pober JS, editors. Antigen receptors and accessory molecules of T lymphocytes; Chap. 6 of Section ii. In: Cellular and molecular immunology. 4th ed. Philadelphia: W. B. Saunders Company; 2000. p. 102–22Google Scholar
  15. 15.
    Bagg A. Immunoglobulin and T-cell receptor gene rearrangements: minding your B’s and T’s in assessing lineage and clonality in neoplastic lymphoproliferative disorders. J Mol Diagnostics. 2006;8:426–9.CrossRefGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2010

Authors and Affiliations

  1. 1.Department of PathologyDuke University Medical CenterDurhamUSA

Personalised recommendations