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γδ T Lymphocytosis Associated with Common Variable Immunodeficiency1

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Abstract

We present the case of a 28-year-old Caucasian female with common variable immunodeficiency (CVID) since age 5 who had a long history of hospitalizations for unexplained fevers and pulmonary infiltrates. The patient developed mild lymphocytosis 7 months prior to our evaluation. Flow cytometry of peripheral blood revealed an expansion of γδ T lymphocytes, mild CD4 T lymphocytopenia, and a reduced CD4/CD8 ratio (0.2). Two subpopulations of γδ T lymphocytes were found (CD3+/CD4−/CD8+, 47%; CD3+/CD4−/CD8−, 53%), the vast majority of which expressed V-δ1. An infectious cause for the patient's γδ T lymphocytosis could not be found. The sputum was chronically colonized with Staphylococcus aureus, and the organism produced TSST-1 in vitro. A bronchoalveolar lavage (BAL) revealed marked lymphocytosis, but γδ T lymphocytes were not overrepresented in the BAL. Lymphocyte functional studies revealed poor proliferative responses to mitogens and staphylococcal superantigens and diminished cytokine production. V-δ1 T lymphocytes from the patient's blood were not expanded in vitro in response to staphylococcal superantigens. TCR gene rearrangement studies confirmed the presence of Jγ and Jβ1 clonal rearrangements accounting for only a small subpopulation of the γδ T lymphocytes. These studies were repeated 5 months later and were unchanged. A bone marrow biopsy was negative for leukemia. Hence, the cause of the patient's γδ T lymphocytosis could not be determined despite evaluation for underlying malignancy, occult infection, or superantigen-driven stimulation. The patient ultimately died of progressive respiratory insufficiency. The state of current knowledge regarding γδ T lymphocytosis, decreased production of αβ T lymphocytes, and a low CD4/CD8 ratio in association with CVID is discussed.

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REFERENCES

  1. Morio T, Nagasawa M, Yata J: Gamma-delta T cells in patients with primary immunodeficiency syndrome: Their function and a possible role in the pathogenesis. Chem Immunol 53:102–120, 1992

    Google Scholar 

  2. Lust JA, Letendre L, Thibodeau SN: Marrow hypoplasia associated with a monoclonal CD8 large granular lymphocyte proliferation: Reversal with cyclophosphamide and prednisone. Am J Med 87:215–217, 1989

    Google Scholar 

  3. Saito H, Kranz DM, Takagaki Y, Hayday AC, Eisen HN, Tonegawa S: Complete primary structure of a heterodimeric T-cell receptor deduced from cDNA sequences. Nature 309:757, 1984

    Google Scholar 

  4. Brenner MB, McLean J, Dialynas DP, Strominger JL, et al.: Identification of a putative second T-cell receptor. Nature 322:145–149, 1986

    Google Scholar 

  5. Parker CM, Veronika G, Hamid B, Porcelli SA, et al.: Evidence for extrathymic changes in the T cell receptor g/d repertoire. J Exp Med 171:1597–1611, 1990

    Google Scholar 

  6. Haas W, Pereira P, Tonegawa S: Gamma/delta cells. Annu Rev Immunol 11:637–685, 1993

    Google Scholar 

  7. Kabelitz D: Function and specificity of human g/d-positive T cells. Crit Rev Immunol 1l(5):281–303, 1992

    Google Scholar 

  8. Scott CS, Richards SJ, Roberts BE: Patterns of membrane TCRab and TCR gd chain expression by normal blood CD4+ CD8−, CD4 CD8+, CD4− CD8drm− and CD4− CD8 lymphocytes. Immunology 70:351–364, 1990

    Google Scholar 

  9. Kabelitz D: Human gd T lymphocytes. Int Arch Allergy Immunol 102:1–9, 1993

    Google Scholar 

  10. Brunangelo F, Flenghi L, Piled S, Pelicci P, et al.: Distribution of T cells bearing different forms of the T cell receptor g/d in normal and pathological human tissues. J Immunol 143:2480–2488, 1989

    Google Scholar 

  11. Saiki O, Ralph P, Cunningham-Rundles C, Good R: Three distinct stages of B-cell defects in common varied immunodeficiency. Proc Natl Acad Sci USA 79:6008–6012, 1982

    Google Scholar 

  12. Farrant J, Bryant A, Almandoz F, Spickett G, Evans SW, Webster ADB: B cell function in acquired common variable hypogammaglobulinemia: Proliferative responses to lymphokines. Clin Immunol Immunopathol 51:196–204, 1989

    Google Scholar 

  13. Wright J, Wagner D, Blaese M, Hagengruber C, Waldmann T, Fleisher T: Characterization of common variable immunodeficiency: Identification of a subset of patients with distinctive immunophenotypic and clinical features. Blood 76:2046–2051, 1990

    Google Scholar 

  14. Loughran T: Clonal diseases of large granular lymphocytes. Blood 82:1–13, 1993

    Google Scholar 

  15. Foroni L, Laffan T, Boehm TH, Rabbitts D, et al.: Rearrangement of the T-cell receptor d genes in human T-cell leukemias. Blood 73:559–565, 1989

    Google Scholar 

  16. Foroni L, Matutes J, Foldi R, Morilla R, et al.: T-cell leukemia with rearrangement of the g but not b T-cell receptor. Blood 71:356–362, 1988

    Google Scholar 

  17. Catovsky D, Matutes E: Leukemias of mature T cells. In: Neoplastic Hematopathology, D Knowles (ed). Baltimore, Williams & Wilkins, 1992, pp 1267–1294.

    Google Scholar 

  18. Sklar J: Leukemias of mature T cells. In Neoplastic Hematopathology, D Knowles (ed). Baltimore, Williams & Wilkins, 1992, pp 1275–1276

    Google Scholar 

  19. Popa V: Lymphocytic interstitial pneumonitis in association with common variable immunodeficiency. Ann Allergy 60:203–206, 1988

    Google Scholar 

  20. Forrester J, Newman L, Wang Y, King T, Kotzin B: Clonal expansion of lung Vdl+ T cells in pulmonary sarcoidosis. J Clin Invest 91:292–300, 1993

    Google Scholar 

  21. Rust CJJ, Koning F: gd T cell reactivity towards bacterial superantigens. Semin Immunol 5:41–46, 1993

    Google Scholar 

  22. Paoli PD, Gennari D, Martelli P, Cavarzerani V, et al.: gd T cell receptor-bearing lymphocytes during Epstein-Barr virus infection. J Infect Dis 161:1013–1016, 1990

    Google Scholar 

  23. Autran B, Triebel F, Katlama C, Rozenbaum W, et al.: T cell receptor g/d+ lymphocyte subsets during HIV infection. Clin Exp Immunol 75:206–210, 1989

    Google Scholar 

  24. Born W, Harshan K, Modlin R, O'Brien R: The role of gd T lymphocytes in infection. Curr Opin Immunol 3:455–459, 1991

    Google Scholar 

  25. Kabelitz D, Bender A, Prospero T, Wesselborg S, Janssen O, Pechold K: The primary response of human gd+ T cells to Mycobacterium tuberculosis is restricted to Vg9-bearing cells. J Exp Med 173:1331–1338, 1991

    Google Scholar 

  26. Morio T, Masayuki N, Shigeaki N, Hiroji O, Yata J: Phenotypic profile and functions of T cell receptor-gd-bearing cells from patients with primary immunodeficiency syndrome. J Immunol 144:1270–1275, 1990

    Google Scholar 

  27. Bonneville M, Ishida I, Mombaerts P, Katsuki M, et al.: Blockage of ab T-cell development by TCR gd transgenes. Nature 342:931–934, 1989

    Google Scholar 

  28. Ishida I, Verbeek S, Bonneville M, Shigeyoshi I, et al.: T-cell receptor gd and g transgenic mice suggest a role of a g gene silencer in the generation of ab T cells. Proc Natl Acad Sci USA 87:3067–3071, 1990

    Google Scholar 

  29. Borowitz MJ, Guenther KL, Shults KE, Stelzer GT: Immunophenotyping of acute leukemia by flow cytometric analysis. Use of CD45 and right-angle light scatter to gate on leukemic blasts in three-color analysis. Am J Clin Pathol 100:534–540, 1993

    Google Scholar 

  30. BAL Cooperative Group Steering Committee: Bronchoalveolar lavage constituents in healthy individuals, idiopathic pulmonary fibrosis, and selected comparison groups. Am Rev Respir Dis 141(5, Pt 2):S169–S202, 1990

    Google Scholar 

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Authors and Affiliations

  1. Department of Allergy and Immunology, Walter Reed Army Medical Center, Washington, DC

    Rohit K. Katial

  2. Department of Clinical Investigations, Fitzsimons Army Medical Center, Aurora, Colorado

    Michael M. Lieberman & Sheri L. Muehlbauer

  3. Mayo Clinic, Division of Hematopathology, Rochester, Minnesota

    J. A. Lust

  4. Division of Allergy and Immunology, Washington University School of Medicine, St. Louis, Missouri

    Daniel L. Hamilos

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  1. Rohit K. Katial
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  2. Michael M. Lieberman
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  3. Sheri L. Muehlbauer
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  4. J. A. Lust
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  5. Daniel L. Hamilos
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Katial, R.K., Lieberman, M.M., Muehlbauer, S.L. et al. γδ T Lymphocytosis Associated with Common Variable Immunodeficiency1 . J Clin Immunol 17, 34–42 (1997). https://doi.org/10.1023/A:1027384311897

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