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Vol. 124, No. 1, 2010
Issue release date: July 2010
Section title: Case Report
Acta Haematol 2010;124:5–8
(DOI:10.1159/000314900)

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

Franco G. · Palazzolo R. · Liardo E. · Tripodo C. · Mancuso S.
aDivision of Hematology with BMT, Department of Oncology, and bDepartment of Human Pathology, University of Palermo, Palermo, Italy

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Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 11/16/2009
Accepted: 3/17/2010
Published online: 5/26/2010

Number of Print Pages: 4
Number of Figures: 2
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

T cell large granular lymphocytic (LGL) leukemia is a rare condition accounting for 2–3% of all mature lymphoid leukemias. Here, we present the case of a 73-year-old woman presenting with neutropenia and anemia (hemoglobin 9.9 g/dl). Hematological assessment revealed the presence of a T cell LGL leukemia. At the time of T cell LGL leukemia diagnosis, the patient developed xerophthalmia and xerostomia, and a diagnosis of Sjögren’s syndrome was made following salivary gland biopsy. The finding of large granular lymphocytes in the context of autoimmune disorders is well-known, though it often occurs with rheumatoid arthritis or in association with a positive autoantibody titer in the absence of an overt clinical picture. The concomitant presentation of T cell LGL leukemia with Sjögren’s syndrome is a rare event which is worth reporting. Our patient was managed with immunosuppressive therapy and is still alive.


Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 11/16/2009
Accepted: 3/17/2010
Published online: 5/26/2010

Number of Print Pages: 4
Number of Figures: 2
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

References

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    External Resources

  7. Loughran TP Jr: Clonal diseases of large granular lymphocytes. Blood 1993;82:1–14.
  8. Viny AD, Lichtin A, Pohlman B, Loughran T, Maciejewski J: Chronic B-cell dyscrasias are an important clinical feature of T-LGL leukemia. Leuk Lymphoma 2008;49:932–938.
  9. Ergas D, Tsimanis A, Shtalrid M, Duskin C, Berrebi A: T-gamma large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren’s syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia. Am J Hematol 2002;69:132–134.
  10. Saitoh T, Matsushima T, Kaneko Y, Yokohama A, Handa H, Tsukamoto N, Karasawa M, Nojima Y, Murukami H: T cell large granular lymphocyte (LGL) leukemia associated with Behcet’s disease: high expression of sFasL and IL-18 of CD8 LGL. Ann Hematol 2008;87:585–586.
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  13. Friedman J, Schattner A, Shvidel L, Berrebi A: Characterization of T-cell large granular lymphocyte leukemia associated with Sjögren’s syndrome – an important but under-recognized association. Semin Arthritis Rheum 2006;35:306–311.