International Journal of Hematology

, Volume 109, Issue 4, pp 499–504 | Cite as

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

  • Wataru KishimotoEmail author
  • Yoko Takiuchi
  • Yoshiki Nakae
  • Sumie Tabata
  • Akiko Fukunaga
  • Naomi Matsuzaki
  • Yoshiaki Yuba
  • Toshiyuki Kitano
  • Nobuyoshi Arima
Case Report


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.


Angioimmunoblastic T-cell lymphoma Peripheral T-cell lymphoma Lenalidomide Plasma cell myeloma 



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

Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.

Supplementary material

12185_2018_2587_MOESM1_ESM.pdf (6.6 mb)
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)


  1. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 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.PubMedGoogle Scholar
  3. 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.CrossRefGoogle Scholar
  4. 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.CrossRefPubMedGoogle Scholar
  5. 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. 6.
    Advani R, Horwitz S, Zelenetz A, Horning SJ. Angioimmunoblastic T cell lymphoma: treatment experience with cyclosporine. Leuk Lymphoma. 2007;48(3):521–5.CrossRefPubMedGoogle Scholar
  7. 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.CrossRefPubMedGoogle Scholar
  8. 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.CrossRefPubMedGoogle Scholar
  9. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 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.CrossRefGoogle Scholar
  12. 12.
    Lunning MA, Vose JM. Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma. Blood. 2017;129(9):1095–102.CrossRefPubMedGoogle Scholar
  13. 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.PubMedGoogle Scholar
  14. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 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.CrossRefPubMedGoogle Scholar
  16. 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.CrossRefPubMedGoogle Scholar
  17. 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.CrossRefPubMedGoogle Scholar
  18. 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.CrossRefPubMedGoogle Scholar
  19. 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.CrossRefPubMedGoogle Scholar

Copyright information

© Japanese Society of Hematology 2019

Authors and Affiliations

  1. 1.Department of HematologyTazuke Kofukai Medical Research Institute, Kitano HospitalOsakaJapan
  2. 2.Department of PathologyTazuke Kofukai Medical Research Institute, Kitano HospitalOsakaJapan

Personalised recommendations