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Selective defect of a T helper subpopulation in severe combined immunodeficiency

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Abstract

The case of an infant female aged 1 month with severe infections, failure to thrive, and skin erythroderma is reported. Immunological studies demonstrated a severe combined immunodeficiency (SCID) with B cells and normal serum IgM levels. Studies of T-cell subpopulations showed a defect of a subset of T helper cells. Possible pathogenetic mechanisms are discussed.

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References

  1. Aiuti F, Businco L, Griscelli C, Touraine J, Webster ADB: Improvements in methods for identifying patients with severe combined immunodeficiency and related syndromes. Z Immunol Forsch 153:95, 1977

    Google Scholar 

  2. Aiuti F, Businco L: Classification and therapy of T-lymphocyte defects.In The Immune System. Function and Therapy of Disfunction, G Doria, A Eshkol (eds). New York, Academic Press, 1980, pp 139–154

    Google Scholar 

  3. Parkman R, Gelfand EW, Rosen FS, Sanderson A, Hirschhorn R: Severe combined immunodeficiency and adenosine deaminase deficiency. N Engl J Med 194:714, 1975

    Google Scholar 

  4. Moretta L, Webb SR, Grossi CE, Lydyard PM, Cooper MD: Functional analysis of two human T-cell subpopulations: Help and suppression of B-cell responses by T cells bearing receptors for IgM or IgG. J Exp Med 146:185, 1977

    Google Scholar 

  5. Evans RL, Lazarus H, Penta AC, Schlossman SF: Two functionally distinct subpopulations of human T cells that collaborate in the generation of cytotoxic cells responsible for cell-mediated lympholysis. J Immunol 120:1423, 1978

    Google Scholar 

  6. Shore A, Dosch HM, Gelfan EW: Induction and separation of antigen-dependent T helper and T suppressor cells in man. Nature 274:587, 1978

    Google Scholar 

  7. Reinherz E, Kung PC, Golstein G, Schlossman SF: Further characterization of the human inducer T cell subset defined by monoclonal antibody. J Immunol 123:2894, 1979

    Google Scholar 

  8. Aiuti F, Pandolfi F: Techniques used for separation and characterization of T and B lymphocytes. Folia Allergol Immunol Clin 24:83, 1977

    Google Scholar 

  9. Limatibul S, Shore A, Dosch HM, Gelfand EW: Theophylline modulation of E-rosette formation: Indicator of T-cell maturation. Clin Exp Immunol 33:503, 1978

    Google Scholar 

  10. Pandolfi F, Strong DM, Slease RB, Bonnard GD: Serological and functional characterization of human T cell subsets. Clin Exp Immunol (in press), 1981

  11. Pandolfi F, Strong DM, Bonnard GD, Heberman RB: Lack of expression on cultured human T-lymphocytes of a T-cell antigen shared by the MOLT-4 cell line and normal human thymocytes. In Vitro 16:754, 1980

    Google Scholar 

  12. Pandolfi F, Nilsson R, Quinti I, Aiuti F: The formation of “stable” rosettes by mitogen activated lymphocytes and hemotopoietic cell lines. Thymus 1:347, 1980

    Google Scholar 

  13. Pandolfi F, Strong DN, Slease RB, Smith ML, Ortaldo JR, Heberman RB: Characterization of a suppressor T-cell chronic lymphocytic leukemia with ADCC but not NK activity. Blood 56:653, 1980

    Google Scholar 

  14. Humphreys RE, McCune J, Chess L, Herrman HC, Malenka DJ, Mann DL, Parham P, Scholssman SF, Strominger JL: Isolation and immunologic characterization of a human, B-lymphocyte-specific, cell surface antigen. J Exp Med 144:89, 1976

    Google Scholar 

  15. Woody JN, Ahmed A, Knudsen RC, Strong DM, Sell KW: Human T cell heterogeneity as delineated with a specific human thymus lymphocyte antiserum. In vitro effects on mitogen response, mixed leukocyte cultures, cell-mediated lymphocytotoxicity and lymphokine production. J Clin Invest 55:956, 1975

    Google Scholar 

  16. Goldstein G, Scheid MP, Boyse EA: A synthetic pentapeptide with biological activity characteristic of the thymic hormone thymopoietin. Science 204:1309, 1979

    Google Scholar 

  17. Aiuti F, Businco L, Rossi P, Quinti I: Response to thymopoietin pentapeptide in patient with DiGeorge syndrome. Lancet 1:91, 1980

    Google Scholar 

  18. Aiuti F, Ammirati P, Fiorilli M, D'Amelio R, Franchi F, Calvani M, Businco L: Immunologic and clinical investigation on a bovine thymic extract. Therapeutic applications in primary immunodeficiencies. Pediatr Res 13:797, 1979

    Google Scholar 

  19. Gatti RA, Platt N, Pomerana NN: Hereditary lymphopenic agammaglobulinemia associated with a distinctive form of short limbed dwarfism and ectodermal dysplasia. J Pediatr 75:675, 1972

    Google Scholar 

  20. Jondal M, Holm G, Wigzell H: Surface markers on human T and B lymphocytes. A large population of lymphocytes forming non-immune rosettes with SRBC. J Exp Med 136:207, 1972

    Google Scholar 

  21. Pandolfi F, Kurnick JT, Nilsson K, Forsbeck K, Wigzell H: Rosette formation with goat erythrocytes. A marker for human T lymphocytes. Clin Exp Immunol 32:504, 1978

    Google Scholar 

  22. Aiuti F, Wigzell H: Function and distribution pattern of human T lymphocytes. I. Production of anti-T lymphocyte specific sera as estimated by cytotoxicity and elimination of function of lymphocytes. Clin Exp Immunol 13:171, 1973

    Google Scholar 

  23. Reinherz EL, Kung PC, Goldstein G, Levey RH, Schlossman SF: Discrete stages of human intrathymic differentiation: Analysis of normal thymocytes and leukemic lymphoblasts of T-cell lineage. Proc Natl Add Sci USA 3:1588, 1980

    Google Scholar 

  24. Buckley RH, Gilbertsen RB, Shiff RI, Ferreira E, Sanal SO, Woldmann TA: Heterogeneity of lymphocyte subpopulations in severe combined immunodeficiency. J Clin Invest 58:130, 1976

    Google Scholar 

  25. Alter BJ, Bach FH: Speculations on alternative pathways of T lymphocyte response. Scand J Immunol 10:87, 1979

    Google Scholar 

  26. Gupta S, Good RA: Human T cell subpopulations as defined by Fc receptors. Thymus 3:135, 1979

    Google Scholar 

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Businco, L., Pandolfi, F., Rossi, P. et al. Selective defect of a T helper subpopulation in severe combined immunodeficiency. J Clin Immunol 1, 125–130 (1981). https://doi.org/10.1007/BF00915390

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  • DOI: https://doi.org/10.1007/BF00915390

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