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