Korean J Hematol.  2008 Sep;43(3):174-178. 10.5045/kjh.2008.43.3.174.

A Case of Epstein-Barr Virus-positive Diffuse, Large B-cell Lymphoma after Angioimmunoblastic T-cell Lymphoma

• Choi YH
• Kim HS
• Nam SB
• Kang HJ
• Na II
• Yang SH
• Ryoo BY

• KMID: 2252185
• DOI: http://doi.org/10.5045/kjh.2008.43.3.174

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a systemic lymphoproliferative disorder that presents with profound immune dysfunction and immunodeficiency. As in many other immunodeficiencies, Epstein-Barr virus (EBV) associated B-cell lymphoid proliferation can occur in AITL but few cases of EBV-positive B-cell lymphoma have been reported in patients with preexisting AITL. We report a case of AITL in which EBV-positive diffuse large B-cell lymphoma (DLBCL) developed 13 months after the initial diagnosis of AITL. Although the exact mechanisms remain unclear, Epstein-Barr virus may have played a role in the pathogenesis of the secondary DLBCL

Keyword

Angioimmunoblastic T-cell lymphoma; Diffuse large B-cell lymphoma; Epstein-Barr virus

MeSH Terms

B-Lymphocytes
Herpesvirus 4, Human
Humans
Lymphoma, B-Cell
Lymphoma, T-Cell
Lymphoproliferative Disorders
T-Lymphocytes

Figure

• 

  Fig. 1 Angioimmunoblastic T cell lymphoma in the cervical lymph node. (A) The nodal architecture was effaced by a diffuse infiltration of medium-sized atypical lymphocytes with clear cytoplasm in a background of arborizing endothelial venules and various-sized admixture (H&E, ×400). (B) Tumor cells were positive for CD3. (C) Tumor cells were positive for CD21.

• 

  Fig. 2 The tonsilar biopsy showed a diffuse large B cell lymphoma that developed 13 months after the diagnosis of angioimmunoblastic T cell lymphoma. (A) Diffuse infiltration of large-sized lymphoid cells were shown (H&E, ×400). (B) Tumor cells were diffusely positive for CD20. (C) In situ hybridization of EBV showed the positivity of a few large lymphocytes.

Reference

1). Dogan A., Attygalle AD., Kyriakou C. Angioimmunoblastic T-cell lymphoma. Br J Haematol. 2003. 121:681–91.

2). Khan G., Norton AJ., Slavin G. Epstein-Barr virus in angioimmunoblastic T-cell lymphomas. Histopathology. 1993. 22:145–9.

3). Weiss LM., Jaffe ES., Liu XF., Chen YY., Shibata D., Medeiros LJ. Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma. Blood. 1992. 79:1789–95.

4). Ohshima K., Takeo H., Kikuchi M, et al. Heterogeneity of Epstein-Barr virus infection in angioimmunoblastic lymphadenopathy type T-cell lymphoma. Histopathology. 1994. 25:569–79.

5). Tan BT., Warnke RA., Arber DA. The frequency of B- and T-cell gene rearrangements and Epstein-Barr virus in T-cell lymphomas: a comparison between angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified with and without associated B-cell proliferations. J Mol Diagn. 2006. 8:466–75.

6). Abruzzo LV., Schmidt K., Weiss LM, et al. B-cell lymphoma after angioimmunoblastic lymphadenopathy: a case with oligoclonal gene rearrangements associated with Epstein-Barr virus. Blood. 1993. 82:241–6.

7). Knecht H., Martius F., Bachmann E, et al. A deletion mutant of the LMP1 oncogene of Epstein-Barr virus is associated with evolution of angioimmunoblastic lymphadenopathy into B immunoblastic lymphoma. Leukemia. 1995. 9:458–65.

8). Matsue K., Itoh M., Tsukuda K., Kokubo T., Hirose Y. Development of Epstein-Barr virus-associated B cell lymphoma after intensive treatment of patients with angioimmunoblastic lymphadenopathy with dysproteinemia. Int J Hematol. 1998. 67:319–29.

9). Zettl A., Lee SS., Rüdiger T, et al. Epstein-Barr virus-associated B-cell lymphoproliferative disorders in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified. Am J Clin Pathol. 2002. 117:368–79.

10). Xu Y., McKenna RW., Hoang MP., Collins RH., Kroft SH. Composite angioimmunoblastic T-cell lymphoma and diffuse large B-cell lymphoma: a case report and review of the literature. Am J Clin Pathol. 2002. 118:848–54.

11). Hawley RC., Cankovic M., Zarbo RJ. Angioimmunoblastic T-cell lymphoma with supervening Epstein-Barr virus-associated large B-cell lymphoma. Arch Pathol Lab Med. 2006. 130:1707–11.

12). Attygalle AD., Kyriakou C., Dupuis J, et al. Histologic evolution of angioimmunoblastic T-cell lymphoma in consecutive biopsies: clinical correlation and insights into natural history and disease progression. Am J Surg Pathol. 2007. 31:1077–88.

13). van Dongen JJ., Wolvers-Tettero IL. Analysis of immunoglobulin and T cell receptor genes. part II: Possibilities and limitations in the diagnosis and management of lymphoproliferative diseases and related disorders. Clin Chim Acta. 1991. 198:93–174.

14). Krenacs L., Schaerli P., Kis G., Bagdi E. Phenotype of neoplastic cells in angioimmunoblastic T-cell lymphoma is consistent with activated follicular B helper T cells. Blood. 2006. 108:1110–1.

15). Grogg KL., Attygalle AD., Macon WR., Remstein ED., Kurtin PJ., Dogan A. Angioimmunoblastic T-cell lymphoma: a neoplasm of germinal-center T-helper cells? Blood. 2005. 106:1501–2.