Skip to main content

Advertisement

SpringerLink
Log in

CD103+ γδ T cell large granular lymphocytosis in a patient with refractory celiac disease: a diagnostic enigma

  • Case Report
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Indolent γδ T cell lymphomas/leukemias are rare and overlap with the morphological spectrum of large granular lymphocyte (LGL) leukemia. We report an extremely rare case of CD103+ γδ T LGL leukemia in a patient with celiac disease who presented with refractory diarrhea. Whether the refractory diarrhea in our patient was a manifestation of LGL leukemia itself or whether the clonal LGL expansion is a manifestation of refractory celiac disease (RCD) remains an enigma. This report highlights the diagnostic difficulties and the need of consensus in categorizing clonal CD103+ lymphocytosis in patients with RCD.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Gaulard P, Belhadj K, Reyes F. Gammadelta T-cell lymphomas. Semin Hematol. 2003;40:233–43.

    Article  PubMed  Google Scholar 

  2. Ahmad E, Kingma DW, Jaffe ES, Schrager JA, Janik J, Wilson W, et al. Flow cytometric immunophenotypic profiles of mature gamma delta T-cell malignancies involving peripheral blood and bone marrow. Cytometry B Clin Cytom. 2005;67:6–12.

    Article  PubMed  Google Scholar 

  3. Rubio-Tapia A. Murray JA, Classification and management of refractory coeliac disease. Gut. 2010;59:547–57.

    Article  PubMed Central  PubMed  Google Scholar 

  4. Uss E, Rowshani AT, Hooibrink B, Lardy NM, van Lier RA, ten Berge IJ. CD103 is a marker for alloantigen-induced regulatory CD8 + T cells. J Immunol. 2006;177:2775–83.

    Article  CAS  PubMed  Google Scholar 

  5. Cerf-Bensussan N, Jarry A, Brousse N, Lisowska-Grospierre B, Guy-Grand D, Griscelli C. A monoclonal antibody (HML-1) defining a novel membrane molecule present on human intestinal lymphocytes. Eur J Immunol. 1987;17:1279–85.

    Article  CAS  PubMed  Google Scholar 

  6. Annacker O, Coombes JL, Malmstrom V, Uhlig HH, Bourne T, Johansson-Lindbom B, et al. Essential role for CD103 in the T cell-mediated regulation of experimental colitis. J Exp Med. 2005;202:1051–61.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  7. Le Floc’h A, Le Jalil A, Vergnon I, Maux Chansac B, Lazar V, Bismuth G, et al. Alpha E beta 7 integrin interaction with E-cadherin promotes antitumor CTL activity by triggering lytic granule polarization and exocytosis. J Exp Med. 2007;204:559–70.

    Article  PubMed Central  PubMed  Google Scholar 

  8. Venkataraman G, Aguhar C, Kreitman RJ, Yuan CM, Stetler-Stevenson M. Characteristic CD103 and CD123 expression pattern defines hairy cell leukemia: usefulness of CD123 and CD103 in the diagnosis of mature B-cell lymphoproliferative disorders. Am J Clin Pathol. 2011;136:625–30.

    Article  PubMed  Google Scholar 

  9. Katoh A, Ohshima K, Kanda M, Haraoka S, Sugihara M, Suzumiya J, et al. Gastrointestinal T cell lymphoma: predominant cytotoxic phenotypes, including alpha/beta, gamma/delta T cell and natural killer cells. Leuk Lymphoma. 2000;39:97–111.

    Article  CAS  PubMed  Google Scholar 

  10. Malamut G, Afchain P, Verkarre V, Lecomte T, Amiot A, Damotte D, et al. Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II. Gastroenterology. 2009;136:81–90.

    Article  PubMed  Google Scholar 

  11. Lopez P, Morris DG, Galbraith PR, Lillicrap DP, Pross HF. Lymphoproliferative disease of “LAK cell” precursor large granular lymphocytes in association with celiac disease. Am J Hematol. 1993;43:116–22.

    Article  CAS  PubMed  Google Scholar 

  12. Molitor JL, Saint-Louis J, Louvet C, Vachon A, Vincent L, Beaulieu R. Large granular T-cell lymphocytic leukemia disclosed by bilateral uveitis: association with celiac disease. Rev Med Interne. 1997;18:237–9.

    Article  CAS  PubMed  Google Scholar 

  13. Malamut G, Meresse B, Verkarre V, Kaltenbach S, Montcuquet N, Duong Van Huyen JP, et al. Large granular lymphocytic leukemia: a treatable form of refractory celiac disease. Gastroenterology. 2012;143:1470–2 e2.

    Article  PubMed  Google Scholar 

  14. Carbonnel F, Grollet-Bioul L, Brouet JC, Teilhac MF, Cosnes J, Angonin R, et al. Are complicated forms of celiac disease cryptic T-cell lymphomas? Blood. 1998;92:3879–86.

    CAS  PubMed  Google Scholar 

  15. Roden AC, Morice WG, Hanson CA. Immunophenotypic attributes of benign peripheral blood gammadelta T cells and conditions associated with their increase. Arch Pathol Lab Med. 2008;132:1774–80.

    PubMed  Google Scholar 

  16. Garcia-Herrera A, Song JY, Chuang SS, Villamor N, Colomo L, Pittaluga S, et al. Nonhepatosplenic gammadelta T-cell lymphomas represent a spectrum of aggressive cytotoxic T-cell lymphomas with a mainly extranodal presentation. Am J Surg Pathol. 2011;35:1214–25.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Bourgault-Rouxel AS, Loughran TP Jr, Zambello R, Epling-Burnette PK, Semenzato G, Donadieu J, et al. Clinical spectrum of gammadelta + T cell LGL leukemia: analysis of 20 cases. Leuk Res. 2008;32:45–8.

    Article  CAS  PubMed  Google Scholar 

  18. Dearden CE, Johnson R, Pettengell R, Devereux S, Cwynarski K, Whittaker S, et al. British Committee for Standards in Haematology. Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma). Br J Haematol. 2011;153:451–85.

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

Authors have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Hematology, Post Graduate Institute of Medical Education and Research (PGIMER), V Floor, SS Anand Block (Research Block A), Chandigarh, 160012, India

    Sreejesh Sreedharanunni, Neelam Varma & Man Updesh Singh Sachdeva

  2. Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Kirti Gupta

  3. Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Rakesh Kochhar

  4. Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Pankaj Malhotra & Subhash Varma

  5. Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, 632004, India

    Rekha Pai

Authors
  1. Sreejesh Sreedharanunni
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Neelam Varma
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Man Updesh Singh Sachdeva
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kirti Gupta
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Rekha Pai
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rakesh Kochhar
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Pankaj Malhotra
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Subhash Varma
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Neelam Varma.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sreedharanunni, S., Varma, N., Sachdeva, M.U.S. et al. CD103+ γδ T cell large granular lymphocytosis in a patient with refractory celiac disease: a diagnostic enigma. Int J Hematol 101, 603–607 (2015). https://doi.org/10.1007/s12185-015-1736-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-015-1736-x

Keywords

Search

Navigation