Skip to main content

Advertisement

Log in

A case of AITL complicated by EBV-positive B cell and monoclonal plasma cell proliferation and effectively treated with lenalidomide

  • Case Report
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a common subtype of peripheral T-cell lymphoma with an aggressive clinical course and poor prognosis after conventional chemotherapy, for which there is no current standard of care. We describe here an 87-year-old woman with AITL, whose clinical diagnosis was complicated by the presence of B immunoblasts positive for Epstein–Barr virus in the lymph nodes and monoclonal plasma cells in the bone marrow at initial presentation. Rebiopsy of the lymph node led to the correct diagnosis of AITL with concurrent smoldering plasma cell myeloma. She was treated with several courses of conventional chemotherapy, resulting in progressive disease, and then switched to the immunomodulatory drug lenalidomide, which used in Japan for the treatment of multiple myeloma. Lenalidomide was effective in controlling both AITL and plasma cell myeloma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Mourad N, Mounier N, Briere J, Raffoux E, Delmer A, Feller A, et al. Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d’Etude des Lymphomes de l’Adulte (GELA) trials. Blood. 2008;111(9):4463–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. 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(7):1789–95.

    CAS  PubMed  Google Scholar 

  3. Xu J, Tang Y, Zhao S, Zhang W, Xiu Y, Liu T, et al. Angioimmunoblastic T-cell lymphoma with coexisting plasma cell myeloma: a case report and review of the literature. Tohuku J Exp Med. 2015;235(4):283–8.

    Article  CAS  Google Scholar 

  4. De Leval L, Gisselbrecht C, Gaulard P. Advances in the understanding and management of angioimmunoblastic T-cell lymphoma. Br J Haematol. 2010;148(5):673–89.

    Article  CAS  PubMed  Google Scholar 

  5. Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S, Stein H, et al. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC; 2017.

    Google Scholar 

  6. Advani R, Horwitz S, Zelenetz A, Horning SJ. Angioimmunoblastic T cell lymphoma: treatment experience with cyclosporine. Leuk Lymphoma. 2007;48(3):521–5.

    Article  CAS  PubMed  Google Scholar 

  7. Zettl A, Lee S-S, Rüdiger T, Starostik P, Marino M, Kirchner 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(3):368–79.

    Article  PubMed  Google Scholar 

  8. Balagué O, Martínez A, Colomo L, Roselló E, Garcia A, Martínez-Bernal M, et al. Epstein–Barr virus negative clonal plasma cell proliferations and lymphomas in peripheral T-cell lymphomas: a phenomenon with distinctive clinicopathologic features. Am J Surg Pathol. 2007;31(9):1310–22.

    Article  PubMed  Google Scholar 

  9. Huppmann AR, Roullet MR, Raffeld M, Jaffe ES. Angioimmunoblastic T-cell lymphoma partially obscured by an Epstein–Barr virus-negative clonal plasma cell proliferation. J Clin Oncol. 2012;31(2):e28–30.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Laichalk LL, Thorley-Lawson DA. Terminal differentiation into plasma cells initiates the replicative cycle of Epstein–Barr virus in vivo. J Virol. 2005;79(2):1296–307.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. de Leval L, Rickman DS, Thielen C, de Reynies A, Huang Y-L, Delsol G, et al. The gene expression profile of nodal peripheral T-cell lymphoma demonstrates a molecular link between angioimmunoblastic T-cell lymphoma (AITL) and follicular helper T (TFH) cells. Blood. 2007;109(11):4952–63.

    Article  CAS  PubMed  Google Scholar 

  12. Lunning MA, Vose JM. Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma. Blood. 2017;129(9):1095–102.

    Article  CAS  PubMed  Google Scholar 

  13. Kyriakou C, Canals C, Goldstone A, Caballero D, Metzner B, Kobbe G, et al. High-dose therapy and autologous stem-cell transplantation in angioimmunoblastic lymphoma: complete remission at transplantation is the major determinant of outcome-lymphoma working party of the European Group of Blood and Marrow Transplantation. J Clin Oncol. 2008;26(2):218–24.

    CAS  PubMed  Google Scholar 

  14. O’Connor OA, Pro B, Pinter-Brown L, Bartlett N, Popplewell L, Coiffier B, et al. Pralatrexate in patients with relapsed or refractory peripheral T-cell lymphoma: results from the pivotal PROPEL study. J Clin Oncol. 2011;29(9):1182.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Coiffier B, Pro B, Prince HM, Foss F, Sokol L, Greenwood M, et al. Results from a pivotal, open-label, phase II study of romidepsin in relapsed or refractory peripheral T-cell lymphoma after prior systemic therapy. J Clin Oncol. 2012;30(6):631–6.

    Article  CAS  PubMed  Google Scholar 

  16. Morschhauser F, Fitoussi O, Haioun C, Thieblemont C, Quach H, Delarue R, et al. A phase 2, multicentre, single-arm, open-label study to evaluate the safety and efficacy of single-agent lenalidomide (Revlimid®) in subjects with relapsed or refractory peripheral T-cell non-Hodgkin lymphoma: The EXPECT trial. Eur J Cancer. 2013;49(13):2869–76.

    Article  CAS  PubMed  Google Scholar 

  17. Querfeld C, Rosen ST, Guitart J, Duvic M, Kim YH, Dusza SW, et al. Results of an open-label multicenter phase 2 trial of lenalidomide monotherapy in refractory mycosis fungoides and Sezary syndrome. Blood. 2014;123(8):1159–66.

    Article  CAS  PubMed  Google Scholar 

  18. Toumishey E, Prasad A, Dueck G, Chua N, Finch D, Johnston J, et al. Final report of a phase 2 clinical trial of lenalidomide monotherapy for patients with T-cell lymphoma. Cancer. 2015;121(5):716–23.

    Article  CAS  PubMed  Google Scholar 

  19. Miyoshi H, Ohshima K. Epidemiology of malignant lymphoma and recent progress in research on adult T-cell leukemia/lymphoma in Japan. Int J Hematol. 2018;107(4):420–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Mr. Masahiro Hirata and Mr. Hiroyuki Shirahase, Department of Diagnostic Pathology, Kyoto University Hospital, for their technical assistance.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Wataru Kishimoto.

Ethics declarations

Conflict of interest

The authors declare no conflict of interest.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Below is the link to the electronic supplementary material.

12185_2018_2587_MOESM1_ESM.pdf

Figure S1 Multiplex PCR assessment of clonal IgH gene rearrangement using DNA extracted from the lymph node biopsied before treatment and from the bone marrow. In both samples, clonal peaks were detected (arrow heads) by capillary electrophoresis of the PCR products amplified with IgH VH (FR1, FR2, and FR3)-JH primer sets. DLBCL, diffuse large B-cell lymphoma; PCM, plasma cell myeloma (PDF 6792 KB)

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kishimoto, W., Takiuchi, Y., Nakae, Y. et al. A case of AITL complicated by EBV-positive B cell and monoclonal plasma cell proliferation and effectively treated with lenalidomide. Int J Hematol 109, 499–504 (2019). https://doi.org/10.1007/s12185-018-02587-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-018-02587-6

Keywords

Navigation