t(8;22)(p11;q11) is a rare but recurrent chromosome translocation that has been reported in 11 cases of myeloproliferative neoplasm or B-acute lymphoblastic leukemia. This translocation results in an in-frame fusion of FGFR1 on 8p11 and BCR on 22q11, and causes constitutive activation of the tyrosine kinase of the BCR/FGFR1 chimera protein. Here, we report the twelfth case of hematological tumor bearing t(8;22)(p11;q11). The bone marrow showed hypoplastic and tri-lineage dysplasia with 24.4% abnormal cells. The abnormal cells were not defined as myeloid or lymphoid morphologically, lacking a myeloperoxidase reaction. Flow cytometric analysis of the bone marrow cells revealed that the abnormal cells expressed CD13, CD33, CD34, and CD19, and that a fraction of the abnormal cells was positive for CD10. Southern blot analysis of the bone marrow cells showed rearrangement of the immunoglobulin heavy chain gene, a genetic hallmark of B-cell differentiation. Previously reported cases with t(8;22)(p11;q11) suggested an association between myeloid and B-lymphoid tumors, whereas other chromosome translocations involving FGFR1 on 8p11 showed a link between myeloid and T-lymphoid tumors. Our observation supports that t(8;22)(p11;q11) might define a dual myeloid and B-lymphoid disorder.

1.
Fioretos T, Panagopoulos I, Lassen C, Swedin A, Billström R, Isaksson M, Strömbeck B, Olofsson T, Mitelman F, Johansson B: Fusion of the BCR and the fibroblast growth factor receptor-1 (FGFR1) genes as a result of t(8;22)(p11;q11) in a myeloproliferative disorder: the first fusion gene involving BCR but not ABL. Genes Chromosomes Cancer 2001;32:302–310.
2.
Demiroglu A, Steer EJ, Heath C, Taylor K, Bentley M, Allen SL, Koduru P, Brody JP, Hawson G, Rodwell R, Doody ML, Carnicero F, Reiter A, Goldman JM, Melo JV, Cross NC: The t(8;22) in chronic myeloid leukemia fuses BCR to FGFR1: transforming activity and specific inhibition of FGFR1 fusion proteins. Blood 2001;98:3778–3783.
3.
Pini M, Gottardi E, Scaravaglio P, Giugliano E, Libener R, Baraldi A, Muzio A, Cornaglia E, Saglio G, Levis A: A fourth case of BCR-FGFR1 positive CML-like disease with t(8;22) translocation showing an extensive deletion on the derivative chromosome 8p. Hematol J 2002;3:315–316.
4.
Murati A, Arnoulet C, Lafage-Pochitaloff M, Adélaide J, Derré M, Slama B, Delaval B, Popovici C, Vey N, Xerri L, Mozziconacci MJ, Boulat O, Sainty D, Birnbaum D, Chaffanet M: Dual lympho-myeloproliferative disorder in a patient with t(8;22) with BCR-FGFR1 gene fusion. Int J Oncol 2005;26:1485–1492.
5.
Agerstam H, Lilljebjörn H, Lassen C, Swedin A, Richter J, Vandenberghe P, Johansson B, Fioretos T: Fusion gene-mediated truncation of RUNX1 as a potential mechanism underlying disease progression in the 8p11 myeloproliferative syndrome. Genes Chromosomes Cancer 2007;46:635–643.
6.
Lee SG, Park TS, Lee ST, Lee KA, Song J, Kim J, Suh B, Choi JR, Park R: Rare translocations involving chromosome band 8p11 in myeloid neoplasms. Cancer Genet Cytogenet 2008;186:127–129.
7.
Richebourg S, Theisen O, Plantier I, Parry A, Soenen-Cornu V, Lepelley P, Preudhomme C, Renneville A, Laï JL, Roche-Lestienne C: Chronic myeloproliferative disorder with t(8;22)(p11;q11) can mime clonal cytogenetic evolution of authentic chronic myelogeneous leukemia. Genes Chromosomes Cancer 2008;47:915–918.
8.
Baldazzi C, Iacobucci I, Luatti S, Ottaviani E, Marzocchi G, Paolini S, Stacchini M, Papayannidis C, Gamberini C, Martinelli G, Baccarani M, Testoni N: B-cell acute lymphoblastic leukemia as evolution of a 8p11 myeloproliferative syndrome with t(8;22)(p11;q11) and BCR-FGFR1 fusion gene. Leuk Res 2010;34:e282–e285.
9.
Wakim JJ, Tirado CA, Chen W, Collins R: t(8;22)/BCR-FGFR1 myeloproliferative disorder presenting as B-acute lymphoblastic leukemia: report of a case treated with sorafenib and review of the literature. Leuk Res 2010;35:e151–e153.
10.
Kim SY, Oh B, She CJ, Kim HK, Jeon YK, Shin MG, Yoon SS, Lee DS: 8p11 myeloproliferative syndrome with BCR-FGFR1 rearrangement presenting with T-lymphoblastic lymphoma and bone marrow stromal cell proliferation: a case report and review of the literature. Leuk Res 2011;35:e30–e34.
11.
Roumiantsev S, Krause DS, Neumann CA, Dimitri CA, Asiedu F, Cross NC, Van Etten RA: Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations. Cancer Cell 2004;5:287–298.
12.
Agerstam H, Järås M, Andersson A, Johnels P, Hansen N, Lassen C, Rissler M, Gisselsson D, Olofsson T, Richter J, Fan X, Ehinger M, Fioretos T: Modeling the human 8p11-myeloproliferative syndrome in immunodeficient mice. Blood 2010;116:2103–2111.
13.
Ren M, Tidwell JA, Sharma S, Cowell JK: Acute progression of BCR-FGFR1 induced murine B-lympho/myeloproliferative disorder suggests involvement of lineages at the pro-B cell stage. PLoS One 2012;7:e38265.
14.
Watanuki J, Hatakeyama K, Sonoki T, Tatetsu H, Yoshida K, Fujii S, Mizutani M, Abo T, Kurimoto M, Matsuoka H, Matsuno F, Nakakuma H: Bone marrow large B cell lymphoma bearing cyclin D3 expression: clinical, morphologic, immunophenotypic, and genotypic analyses of seven patients. Int J Hematol 2009;90:217–225.
15.
Macdonald D, Reiter A, Cross NC: The 8p11 myeloproliferative syndrome: a distinct clinical entity caused by constitutive activation of FGFR1. Acta Haematol 2002;107:101–107.
16.
Kawamoto H, Katsura Y: A new paradigm for hematopoietic cell lineages: revision of the classical concept of the myeloid-lymphoid dichotomy. Trends Immunol 2009;30:193–200.
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.