Background: Chronic myeloid leukemia (CML) is usually diagnosed in the chronic phase. An extramedullary blast crisis mimicking a T-cell lymphoma is a rare finding. Case Report: A 35-year-old man presented with multiple lymphadenopathy 2 months after diagnosis of Philadelphia chromosome (Ph)-positive CML in the chronic phase. Cervical lymph node biopsy later indicated an extramedullary blast crisis resembling T-cell lymphoblastic lymphoma (TLBL). The tumor mass was composed of primitive lymphoid cells expressing terminal deoxynucleotidyl transferase (TdT), CD3, CD43, CD5, CD99, and Bcl-2. Although the pathological diagnosis, confirmed by 2 independent pathological centers, was more typical of TLBL, fluorescence in situ hybridization (FISH) analysis showed the bcr-abl fusion gene within the blastic tumor cells. The FISH finding confirmed that the mass represented an extramedullary, immature blastic transformation of CML rather than a de novo T-cell lymphoma. Conclusion: The diagnosis of de novo TLBL should be suspected before excluding the extramedullary blast phase of CML.

Dorfman DM, Longtine JA, Fox EA, Weinberg DS, Pinkus GS: T-cell blast crisis in chronic myelogenous leukemia. Immunophenotypic and molecular biologic findings. Am J Clin Pathol 1997; 107:168-176.
Raanani P, Trakhtenbrot L, Rechavi G, Rosenthal E, Avigdor A, Brok-Simoni F, Leiba M, Amariglio N, Nagler A, Ben-Bassat I: Philadelphia-chromosome-positive T-lymphoblastic leukemia: acute leukemia or chronic myelogenous leukemia blastic crisis. Acta Haematol 2005;113:181-189.
Sun T, Susin M, Koduru P, Coffey EL Jr, Dittmar K, Weiss R, Goh J, Brody J: Extramedullary blast crisis in chronic myelogenous leukemia. Demonstration of T-cell lineage and Philadelphia chromosome in a paraspinal tumor. Cancer 1991; 68:605-610.
Ye CC, Echeverri C, Anderson JE, Smith JL, Glassman A, Gulley ML, Claxton D, Craig FE: T-cell blast crisis of chronic myelogenous leukemia manifesting as a large mediastinal tumor. Hum Pathol 2002;33:770-773.
Talvalkar GV, Srinivasan U, Advani SH, Khare AG: Extramedullary blast crisis in chronic myeloid leukaemia simulating lymphoma. Indian J Cancer 1977;14:272-277.
Terjanian T, Kantarjian H, Keating M, Talpaz M, McCredie K, Freireich EJ: Clinical and prognostic features of patients with Philadelphia chromosome-positive chronic myelogenous leukemia and extramedullary disease. Cancer 1987;59:297-300.
Taylor JJ, Evans DW: Lymphosarcoma during the course of myeloid leukaemia. Postgrad Med J 1968;44:558-562.
Kantarjian HM, Keating MJ, Talpaz M, Walters RS, Smith TL, Cork A, McCredie KB, Freireich EJ: Chronic myelogenous leukemia in blast crisis. Analysis of 242 patients. Am J Med 1987;83:445-454.
Specchia G, Palumbo G, Pastore D, Mininni D, Mestice A, Liso V: Extramedullary blast crisis in chronic myeloid leukemia. Leuk Res 1996; 20:905-908.
Kell J, Booth M, Jones B, Appleton M, Lim S: Extramedullary T lymphoblastic transformation of chronic myeloid leukaemia occurring after double allogeneic transplantation. Bone Marrow Transplant 1998;22:813-816.
Gonzalez FA, Villegas A, Ferro MT, Cabello P, Morales D, Perez J, Martinez R: Usefulness of the rearrangement of the bcr/abl gene in extramedullary (lymph nodes) blast crisis diagnosed in chronic myeloid leukaemia. Br J Haematol 1993;84:351-352.
Leone G, La Rocca LM, Teofili L, De Candia E, Landolfi R, Sica S, Zini G, Zollino M, Tabilio A: Lymph node blast crisis in chronic myeloid leukemia mimicking T-immunoblastic lymphoma. Haematologica 1992;77:311-314.
Tittley P, Trempe JM, van der Jagt R, Drouin J, Huebsch L, McLeish B, Cheng G: Occurrence of T-cell lymphoma in a patient with Philadelphia chromosome-positive chronic myelogenous leukemia with rearrangements of BCR and TCR-beta genes in the lymph nodes. Am J Hematol 1993;42: 229-230.
Van Dorpe J, Van Damme S, Jacobs V, Van den Berghe H, Criel A, Michielssen P, Louwagie A: T-lymphoid extramedullary (lymphadenopathic) blast crisis in CML. Acta Clin Belg 1995;50:121-125.
Ganessan K, Goel R, Kumar K, Bakhshi S: Biphenotypic extramedullary blast crisis as a presenting manifestation of Philadelphia chromosome-positive CML in a child. Pediatr Hematol Oncol 2007; 24:195-198.
Jacob A, Rowlands DC, Patton N, Holmes JA: Chronic granulocytic leukaemia presenting with an extramedullary T lymphoblastic crisis. Br J Haematol 1994;88:435-436.
Kim AS, Goldstein SC, Luger S, Van Deerlin VM, Bagg A: Sudden extramedullary T-lymphoblastic blast crisis in chronic myelogenous leukemia: a nonrandom event associated with imatinib? Am J Clin Pathol 2008;129:639-648.
Ichinohasama R, Miura I, Takahashi N, Sugawara T, Tamate E, Endoh K, Endoh F, Naganuma H, DeCoteau JF, Griffin JD, Kadin ME, Ooya K: Ph-negative non-Hodgkin's lymphoma occurring in chronic phase of Ph-positive chronic myelogenous leukemia is defined as a genetically different neoplasm from extramedullary localized blast crisis: report of two cases and review of the literature. Leukemia 2000;14:169-182.
Montefusco E, Lo Coco F, Burgio VL, Rondinelli B, Di Giorgio G, Mancini M, Diverio D, Andriani A, Avvisati G, Alimena G: Occurrence of a Ki-1-positive anaplastic large-cell lymphoma in a patient with Ph' positive chronic myelogenous leukemia successfully treated by alpha-interferon. Leukemia 1993;7:1896-1899.
Rodler E, Welborn J, Hatcher S, Unger K, Larkin E, Gumerlock PH, Wun T, Richman C: Blastic mantle cell lymphoma developing concurrently in a patient with chronic myelogenous leukemia and a review of the literature. Am J Hematol 2004;75:231-238.
Giles FJ, Cortes JE, Kantarjian HM, O'Brien SM: Accelerated and blastic phases of chronic myelogenous leukemia. Hematol Oncol Clin North Am 2004;18:753-774, xii.
Ravandi F, O'Brien S, Thomas D, Faderl S, Jones D, Garris R, Dara S, Jorgensen J, Kebriaei P, Champlin R, Borthakur G, Burger J, Ferrajoli A, Garcia-Manero G, Wierda W, Cortes J, Kantarjian H: First report of phase 2 study of dasatinib with hyper-CVAD for the frontline treatment of patients with Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia. Blood 2010; 116:2070-2077.
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.