Low-grade follicular lymphomas are genetically characterized by the translocation t(14; 18)(q32;q21) with BCL2 gene rearrangements. Marginal zone lymphomas are often associated with translocations or transcriptional deregulations of the MALT gene. We report 2 cases of lymphomas which harbor both the t(14;18)(q32;q21) translocation and MALT gene upregulation. Patients presented with numerous circulating atypical lymphocytes. Lymph node biopsy in both cases on HE staining demonstrated vague nodularity readily highlighted by CD10, CD23, or BCL6. Staining with CD20 and BCL2 demonstrated monotonous diffuse effacement of normal architecture with tumor cells without obvious follicular structures. Morphologically, tumor cells were consistent with centrocytes. Bone marrow biopsy demonstrated a combined peritrabecular and interstitial distribution of the tumor cells. These cases present substantial difficulties for diagnosis and classification. Clinical and morphological features were mostly consistent with follicular lymphoma, with a few features more often seen in marginal zone lymphomas (leukemic presentation, no CD10 in circulating cells, interstitial location of tumor cells in bone marrow); therefore, these cases were finally classified as follicular lymphoma grade I. Both patients were treated with standard chemotherapy regimens for follicular and nongastric MALT lymphomas with a good response to date.

Chang QA, Qasem A, Madhusudhana S, Glazyrin A: The t(14; 18)(q32;q21) with extra MYC signal – is it a gray zone lymphoma? Int J Clin Exp Pathol 2015; 8: 9602–9608.
Streubel B, Simonitsch-Klupp I, Müllauer L, Lamprecht A, Huber D, Siebert R, Stolte M, Trautinger F, Lukas J, Püspök A, Formanek M, Assanasen T, Müller-Hermelink HK, Cerroni L, Raderer M, Chott A: Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. Leukemia 2004; 18: 1722–1726.
Green TM, Young KH, Visco C, Xu-Monette ZY, Orazi A, Go RS, Nielsen O, Gadeberg OV, Mourits-Andersen T, Frederiksen M, Pedersen LM, Møller MB: Immunohistochemical double-hit score is a strong predictor of outcome in patients with diffuse large B-cell lymphoma treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. J Clin Oncol 2012; 30: 3460–3467.
Zinzani PL, Matsuda I, Shimizu Y, Okamoto T, Hirota S: Follicular lymphoma mimicking marginal zone lymphoma in lymph node: a case report. Int. J Clin Exp Pathol 2014; 7: 7076–7081.
Miyaoka M, Kikuchi T, Carreras J, Yara Kikuti Y, Omachi K, Kojima M, Ando K, Nakamura N: Composite follicular lymphoma and CD5-positive nodal marginal zone lymphoma. J Clin Exp Hematop 2016; 56: 55–58.
Goodlad JR, Batstone PJ, Hamilton D, Hollowood K: Follicular lymphoma with marginal zone differentiation: cytogenetic findings in support of a high-risk variant of follicular lymphoma. Histopathology 2003; 42: 292–298.
Torlakovic EE, Aamot HV, Heim S: A marginal zone phenotype in follicular lymphoma with t(14; 18) is associated with secondary cytogenetic aberrations typical of marginal zone lymphoma. J Pathol 2006; 209: 258–264.
Matsuda I, Shimizu Y, Okamoto T, Hirota S: Follicular lymphoma mimicking marginal zone lymphoma in lymph node: a case report. Int J Clin Exp Pathol 2014; 7: 7076–7081.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds): WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, ed 4. Geneva, WHO, 2008.
Zucca E, Conconi A, Pedrinis E, Cortelazzo S, Motta T, Gospodarowicz MK, Patterson BJ, Ferreri AJ, Ponzoni M, Devizzi L, Giardini R, Pinotti G, Capella C, Zinzani PL, Pileri S, López-Guillermo A, Campo E, Ambrosetti A, Baldini L, Cavalli F; International Extranodal Lymphoma Study Group: Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood 2003; 101: 2489–2495.
Federico M, Bellei M, Marcheselli L, Luminari S, Lopez-Guillermo A, Vitolo U, Pro B, Pileri S, Pulsoni A, Soubeyran P, Cortelazzo S, Martinelli G, Martelli M, Rigacci L, Arcaini L, Di Raimondo F, Merli F, Sabattini E, McLaughlin P, Solal-Céligny P: Follicular lymphoma international prognostic index 2: a new prognostic index for follicular lymphoma developed by the international follicular lymphoma prognostic factor project. J Clin Oncol 2009; 27: 4555–4562.
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.