Skip to main content
SpringerLink
Log in

Lymphocytosis of large granular lymphocytes associated with anemia and neutropenia: Proof of monoclonality of the LGL-population, but benign clinical course

  • Kasuistiken
  • Published:
Klinische Wochenschrift Aims and scope Submit manuscript

Summary

A 37-year-old man with severe transfusion-requiring anemia and neutropenia associated with lymphocytosis of large granular lymphocytes (LGLs) has been followed over a period of 3.5 years. Detailed phenotyping of the LGL has been performed, revealing a phenotype of CD3+, CD8+, CD16+, HLA-DR+, and Leu-7+. Southern-blot analysis of the T-cell receptor beta-chain locus detected a gene rearrangement, thus providing proof of monoclonality of the peripheral blood LGLs.

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

Similar content being viewed by others

Abbreviations

CD:

Cluster of differentiation

LGLs:

Large granular lymphocytes

PBMCs:

Peripheral blood mononuclear cells

References

  1. Aisenberg AC, Wilkes BM, Harris NL, Ault KA, Darey RW (1981) Chronic T-cell lymphocytosis with neutropenia: report of a case studied with monoclonal antibody. Blood 58:818–822

    Google Scholar 

  2. Bacigalupo A, Podesta M, Mingari MC, Moretta L, Piaggio G, Van Lint MT, Durando A, Marmont AM (1981) Generation of CFU-C/suppressor T-cells in vitro: an experimental model for immune-mediated marrow failure. Blood 57:491–497

    Google Scholar 

  3. Bassen R, Marini B, Allavena P et al. (1987) Raeb in a patient with chronic granulated T-lymphocytosis. Br J Haematol 65:503–504

    Google Scholar 

  4. Berliner N, Dub AD, Linch DC et al. (1986) T-cell receptor gene rearrangements define a monoclonal T-cell proliferation in patients with T-cell lymphocytosis and cytopenia. Blood 67:914–918

    Google Scholar 

  5. Callard RE, Smith CM, Worman C, Linch D, Cawley JC, Beverly PCL (1981) Unusual phenotype and function of an expanded subpopulation of T-cells in patients with haemopoetic disorders. Clin Exp Immunol 43:497–505

    Google Scholar 

  6. Fernandez LA, Pope B, Lee C et al. (1986) Aggressive natural killer cell leukemia in an adult with establishment of an NK cell line. Blood 67:925–930

    Google Scholar 

  7. Foa R, Pier-Giuseppe P, Migone N et al. (1986) Analysis of T-cell receptor beta chain (T β ) gene rearrangement demonstrates the monoclonal nature of T-cell chronic lymphoproliferative disorders. Blood 67:247–250

    Google Scholar 

  8. Freimark B, Lanier L, Phillips J et al. (1987) Comparison of T-cell receptor gene rearrangement in patients with large granular T-cell leucemia and Felty's syndrome. J Immunol 138:1724–1729

    Google Scholar 

  9. Hansson M, Beran M, Andersson B, Kiessling R (1982) Inhibition of in vitro granulopoiesis by autologous allogeneic human NK cells. J Immunol 129:126–131

    Google Scholar 

  10. Herrmann F, Sieber F, Komischke B et al. (1983) Expanded Ty population with the morphology of large granular lymphocytes. I. Immunological, clinical and morphological characterization. Leukem Res 7:667–689

    Google Scholar 

  11. Huhn D (1986) Neue Organelle in peripheren Lymphozyten? Dtsch med Wochenschr 93:2099–2100

    Google Scholar 

  12. Huhn D, Huber C, Gastl G (1982) Large granular lymphocytes: morphological studies. Eur J Immunol 12:985–988

    Google Scholar 

  13. Kasehara T, Dien JY, Dougherty SF (1983) Capacity of human large granular lymphocytes to produce multiple lymphokines. J Immunol 131:2379–2385

    Google Scholar 

  14. Koizumi S, Seki H, Tachinami T et al. (1986) Malignant clonal expansion of large granular lymphocytes with a Leu-11+, Leu-7-surface phenotype: in vitro responsiveness of malignant cells to recombinant human interleukin 2. Blood 68:1065–1073

    Google Scholar 

  15. Lauria F, Foa R, Migone N et al. (1987) Heterogeneity of large granular lymphocyte proliferations: morphological, immunological and molecular analysis in seven patients. Br J Haematol 66:187–191

    Google Scholar 

  16. Loughran TP Jr, Kadin ME, Starkebaum G et al. (1985) Leukemia of large granular lymphocytes: association with clonal chromosomal abnormalities and autoimmune neutropenia, thrombopenia, and haemolytic anemia. Ann intern Med 102:169–175

    Google Scholar 

  17. Loughran TP, Starkebraum G (1987) Large granular lymphocyte leukemia: report of 38 cases and review of the literature. Medicine 66(5):397–405

    Google Scholar 

  18. McKenna RW, Arthur DC, Gajl-Peczalska KJ et al. (1985) Granulated T-cell lymphocytosis with neutropenia: malignant or benign chronic lymphoproliferative disorders. Blood 66:259–266

    Google Scholar 

  19. Nagasawa T, Abe T, Nakagawa T (1981) Pure red cell aplasia and hypogammaglobulinemia associated with T-cell chronic lymphocytic leukemia. Blood 57:1025–1031

    Google Scholar 

  20. O'Connor NTJ, Wainscoat JS, Weatherall DJ, Gatter KC, Feller AC, Isaacson P, Jones D, Lennert K, Pallesen G, Ramsey A, Stein H, Wright DH, Mason DY (1985) Rearrangement of the T-cell receptor beta chain gene in the diagnosis of lymphoproliferative disorders. Lancet I:1295

    Google Scholar 

  21. Pandolfi F, Pezzutto A, De Rossi G et al. (1984) Characterization of two patients with lymphomas of large granular lymphocytes. Cancer 53:445–452

    Google Scholar 

  22. Pistoia V, Ghio R, Nocera A, Perata A, Ferrarini M (1985) Large granular lymphocytes have a promoting activity on human peripheral blood erythroid burst-forming units. Blood 65:464–472

    Google Scholar 

  23. Pistoia V, Prasthofer EF, Tilden AB et al. (1986) Large granular lymphocytes from patients with expanded LGL populations acquire cytoxic functions and release lymphokines upon in vitro activation. Blood 68:1095–1100

    Google Scholar 

  24. Platanias L, Raefsky E, Young N (1987) Neutropenia associated with large granular lymphocytes responsive to corticosteroids in vitro and in vivo. Eur J Haematol 38:89–94

    Google Scholar 

  25. Reynolds CW, Foon KA (1984) Review: Ty-lymphoproliferative disease and related disorders in human and experimental animals: a review of the clinical, cellular and functional characteristics. Blood 64:1146–1158

    Google Scholar 

  26. Serke S, Serke M, Brudler O (1987) Lymphocyte activation by phytohaemagglutinin and pokeweed mitogen. J Immunol Meth 99:167–172

    Google Scholar 

  27. Starkebaum G, Martin PJ, Singer JW et al. (1983) Chronic lymphocytosis with neutropenia: evidence for a novel, abnormal T-cell population associated with antibody-mediated neutrophil destruction. Clin Immunol Immunopathol 27:110–123

    Google Scholar 

  28. Veen van der JPW, Goldschmeding R, Miedema F et al. (1986) K-cell lymphocytosis/neutropenia syndrome: the neutropenia is not caused by autoimmunity. Br J Haematol 64:777–787

    Google Scholar 

  29. Winton EF, Chan WC, Check I et al. (1986) Spontaneous regression of a monoclonal proliferation of large granular lymphocytes associated with reversal of anemia and neutropenia. Blood 67:1427–1432

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Abteilung für Innere Medizin und Poliklinik m.S. Hämatologie und Onkologie, Universitätsklinikum Rudolf Virchow der Freien Universität Berlin, Standort Charlottenburg, Germany

    S. Serke, A. Neubauer & D. Huhn

Authors
  1. S. Serke
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Neubauer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. D. Huhn
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Serke, S., Neubauer, A. & Huhn, D. Lymphocytosis of large granular lymphocytes associated with anemia and neutropenia: Proof of monoclonality of the LGL-population, but benign clinical course. Klin Wochenschr 67, 588–591 (1989). https://doi.org/10.1007/BF01721686

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01721686

Key words

Search

Navigation