Angioimmunoblastic T cell lymphoma (AITL) is a rare subtype of T cell non-Hodgkin lymphoma. The standard therapeutic strategy for AITL has not yet been established, and its prognosis remains poor. This report concerns the effect of cyclosporin A (CsA) on chemotherapy-refractory AITL. A 68-year-old female with AITL with systemic symptoms, such as high fever, skin rash andgeneralized lymphadenopathy, was initially treated with conventional cytotoxic chemotherapies using alkylators, anthracyclines and corticosteroids, which failed to induce remission. However, CsA (4 mg/kg/day) plus dexamethasone treatment resulted in a dramatic regression of the tumors and amelioration of systemic symptoms and induced complete remission (CR) within 2 weeks. Currently, the patient’s CR has continued for more than 18 months with CsA maintenance therapy. Our experience and previously reported findings suggest that CsA may constitute an alternative treatment option for AITL, even though the use of conventional cytotoxic chemotherapy continues to be the first-line therapy on an empirical basis.

Keywords:
Angioimmunoblastic T cell lymphoma, Cyclosporin A, Chemotherapy
1.
Iannitto E, Ferreri A, Minardi V, Tripodo C, Kreipe H: Angioimmunoblastic T-cell lymphoma. Crit Rev Oncol Hematol 2008;68:264–271.
2.
Reiser M, Josting A, Soltani M, Staib P, Salzberger B, Diehl V, Engert A: T-cell non-Hodgkin’s lymphoma in adults: clinicopathological characteristics, response to treatment and prognostic factors. Leuk Lymphoma 2002;43:805–811.
3.
Kyriakou C, Canals C, Goldstone A, Caballero D, Metzner B, Kobbe G, Kolb HJ, Kienast J, Reimer P, Finke J, Oberg G, Hunter A, Theorin N, Sureda A, Schmitz N: 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 for Blood and Marrow Transplantation. J Clin Oncol 2008;26:218–224.
4.
Corradini P, Dodero A, Zallio F, Caracciolo D, Casini M, Bregni M, Narni F, Patriarca F, Boccadoro M, Benedetti F, Rambaldi A, Gianni AM, Tarella C: Graft-versus-lymphoma effect in relapsed peripheral T-cell non-Hodgkin’s lymphomas after reduced-intensity conditioning followed by allogeneic transplantation of hematopoietic cells. J Clin Oncol 2004;22:2172–2176.
5.
Kyriakou C, Canals C, Finke J, Kobbe G, Harousseau JL, Kolb HJ, Novitzky N, Goldstone AH, Sureda A, Schmitz N: Allogeneic stem cell transplantation is able to induce long-term remissions in angioimmunoblastic T-cell lymphoma: a retrospective study from the lymphoma working party of the European group for blood and marrow transplantation. J Clin Oncol 2009;27:3951–3958.
6.
Siegert W, Nerl C, Meuthen I, Zahn T, Brack N, Lennert K, Huhn D: Recombinant human interferon-α in the treatment of angioimmunoblastic lymphadenopathy: results in 12 patients. Leukemia 1991;5:892–895.
7.
Strupp C, Aivado M, Germing U, Gattermann N, Haas R: Angioimmunoblastic lymphadenopathy (AILD) may respond to thalidomide treatment: two case reports. Leuk Lymphoma 2002;43:133–137.
8.
Ramasamy K, Lim Z, Pagliuca A, Salisbury JR, Mufti GJ, Devereux S: Successful treatment of refractory angioimmunoblastic T-cell lymphoma with thalidomide and dexamethasone. Haematologica 2006;91:e117–e118.
9.
Gallamini A, Zaja F, Patti C, Billio A, Specchia MR, Tucci A, Levis A, Manna A, Secondo V, Rigacci L, Pinto A, Iannitto E, Zoli V, Torchio P, Pileri S, Tarella C: Alemtuzumab (Campath-1H) and CHOP chemotherapy as first-line treatment of peripheral T-cell lymphoma: results of a GITIL (Gruppo Italiano Terapie Innovative nei Linfomi) prospective multicenter trial. Blood 2007;110:2316–2323.
10.
Advani R, Horwitz S, Zelenetz A, Horning SJ: Angioimmunoblastic T cell lymphoma: treatment experience with cyclosporine. Leuk Lymphoma 2007;48:521–525.
11.
Murayama T, Imoto S, Takahashi T, Ito T, Matozaki S, Nakagawa T: Successful treatment of angioimmunoblastic lymphadenopathy with dysproteinemia with cyclosporin A. Cancer 1992;69:2567–2570.
12.
Takemori N, Kodaira J, Toyoshima N, Sato T, Sakurai H, Akakura N, Kimura S, Katagiri M: Successful treatment of immunoblastic lymphadenopathy-like T-cell lymphoma with cyclosporin A. Leuk Lymphoma 1999;35:389–395.
13.
Takemori N, Kodaira J, Sato T, Sakurai H, Akakura N, Kimura S, Katagiri M: Successful treatment of IBL-like T-cell lymphoma with cyclosporin A: two case reports with special reference to serum cytokine levels. Hum Cell 2000;13:35–42.
14.
Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS: Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers. Am J Surg Pathol 1998;22:643–655.
15.
Rojnuckarin P, Nakorn TN, Assanasen T, Wannakrairot P, Intragumtornchai T: Cyclosporin in subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma 2007;48:560–563.
16.
Tsukamoto Y, Katsunobu Y, Omura Y, Maeda I, Hirai M, Teshima H, Konishi Y, Inoue T, Sato T. Subcutaneous panniculitis-like T-cell lymphoma: successful initial treatment with prednisolone and cyclosporine A. Intern Med 2006;45:21–24.
17.
Al Zolibani AA, Al Fawzan S, Al Robaee AA, Qureshi MG, Al Nosian H: Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome successfully treated with cyclosporine A. Skinmed 2006;5:195–197.
18.
Mizutani S, Kuroda J, Shimura Y, Kobayashi T, Tsutsumi Y, Yamashita M, Yamamoto M, Ohshiro M, Sasaki N, Kiyota M, Nakayama R, Uchiyama H, Matsumoto Y, Horiike S, Nakamura S, Taniwaki M: Cyclosporine A for chemotherapy-resistant subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome. Acta Haematol 2011;126:8–12.
19.
Cooper DL, Braverman IM, Sarris AH, Durivage HJ, Saidman BH, Davis CA, Hait WN: Cyclosporine treatment of refractory T-cell lymphomas. Cancer 1993;71:2335–2341.
20.
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;106:1501–1502.
21.
Grogg KL, Attygalle AD, Macon WR, Remstein ED, Kurtin PJ, Dogan A: Expression of CXCL13, a chemokine highly upregulated in germinal center T-helper cells, distinguishes angioimmunoblastic T-cell lymphoma from peripheral T-cell lymphoma, unspecified. Mod Pathol 2006;19:1101–1107.
22.
Dunleavy K, Wilson WH: Angioimmunoblastic T-cell lymphoma: immune modulation as a therapeutic strategy. Leuk Lymphoma 2007;48:449–451.
23.
Uno M, Tsuchiyama J, Moriwaki A, Noguchi T, Mizoguchi K, Ogino T, Yoshino T, Okada S, Harada M: In vitro induction of apoptosis for nasal angiocentric natural killer cell lymphoma-derived cell lines, NK-YS, by etoposide and cyclosporine A. Br J Haematol 2001;113:1009–1014.
24.
Medyouf H, Alcalde H, Berthier C, Guillemin MC, dos Santos NR, Janin A, Decaudin D, de Thé H, Ghysdael J: Targeting calcineurin activation as a therapeutic strategy for T-cell acute lymphoblastic leukemia. Nat Med 2007;13:736–741.
© 2011 S. Karger AG, Basel
2011
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.