Abstract
We investigated the prevalence of phenotypically immature IgG B cells (i.e., coexpressing surface IgG and IgM) in the peripheral blood of 12 patients with hypogammaglobulinemia and in normal individuals. Patients had ataxia-telangiectasia (N=1), hyper-IgM combined immunodeficiency (N=1), or common variable immunodeficiency (CVI). IgG/IgM-positive B cells were evaluated by two-color immunofluorescence using fluoresceinor rhodamine-conjugated goat antiserum; to minimize artifacts due toin vivo cytophilic binding of autologous IgG, cell-bound cytophilic Ig were eluted at pH 4 and Fc receptors were blocked by heat-aggregated rabbit IgG before fluorescent staining. All patients, except two with late-onset CVI, had markedly increased proportions of double-stained IgG B cells (56 to 100% of IgG-bearing B cells) in comparison with normal individuals (11 to 33%).
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References
Pearl ER, Vogler LB, Okos AJ, Crist WM, Lawton AR, Cooper MD: B lymphocyte precursors in human bone marrow: An analysis of normal individuals and patients with antibody deficient states. J Immunol 120:1169–1175, 1978
Levitt D, Haber P, Rich K, Cooper MD: Hyper IgM immunodeficiency: A primary defect of B lymphocyte isotype switching. J Clin Invest 72:1650–1656, 1983
Cooper MD, Lawton AR, Backman DE: Agammaglobulinemia with B lymphocytes: Specific defect of plasmacell differentiation. Lancet 2:791–794, 1971
Siegal FP, Pernis B, Kunkel HG: Lymphocytes in human immunodeficiency states: A study of membrane associated immunoglobulins. Eur J Immunol 1:482–486, 1971
Preud'homme JL, Griscelli C, Seligmann M: Immunoglobulins on the surface of lymphocytes in fifty patients with primary immunodeficiency diseases. Clin Immunol Immunopathol 1:241–256, 1973
Waldmann TA, Broder S, Blaese RM, Durm M, Blackman M, Strober W: Role of suppressor T cells in the pathogenesis of common variable hypogammaglobulinemia. Lancet 2:609–613, 1974
Siegal FP, Siegal M, Good RA: Role of helper, suppressor and B cell defects in the pathogenesis of the hypogammaglobulinemias. N Engl J Med 299:172–178, 1978
Rosen FS, Wedgwood RJ, Aiuti F,et al.: Primary immunodeficiency diseases: Report prepared for the WHO by a Scientific Group on Immunodeficiency. Clin Immunol Immunopathol 28:450–475, 1983
Rosen FS, Cooper MD, Wedgwood RJ: The primary immunodeficiencies. N Engl J Med 311:235–242, 1984
Conley ME, Cooper MD: Immature IgA B cells in IgA deficient patients. N Engl J Med 305:495–497, 1981
Haber PL, Kubagawa H, Cooper MD: Epstein-Barr virus-induced immunoglobulin synthesis by B cells from individuals with late-onset panhypogammaglobulinemia. J Clin Immunol 3:253–259, 1983
Mayumi M, Kuritani T, Kubagawa H, Cooper MD: IgG subclass expression by human B lymphocytes and plasma cells: B lymphocytes precommitted to IgG subclass can be preferentially induced by policlonal mitogens with T cell help. J Immunol 130:671–677, 1983
Fiorilli M, Businco L, Pandolfi F, Paganelli R, Russo G, Aiuti F: Heterogeneity of immunological abnormalities in ataxia-telangiectasia. J Clin Immunol 3:135–141, 1983
Reinherz EL, Cooper MD, Schlossman SF, Rosef FS: Abnormalities in T cell maturation and regulation in human beings with immunodeficiency disorders. J Clin Invest 68:699–705, 1981
Aiuti F, Pandolfi F, Fiorilli M, Bonomo R, Quinti I, Frielingsdorf A, Luzi G: Monoclonal antibody analysis of T cell subsets in 40 patients with immunodeficiencies. J Clin Immunol 2 (Suppl):81–89, 1982
Siegal FP, Siegal M, Good RA: Suppression of B-cell differentiation by leukocytes from hypogammaglobulinemic patients. J Clin Invest 58:109–122, 1976
Durandy A, Griscelli C: Activation par les gammaglobulines injectables des fonctions suppressives de la response humorale testee in vitro. Presse Med 12:2567–2571, 1983
Siegal FP: IgG on infants' B lymphocytes: Enhanced binding of IgG by IgM-bearing lymphoid cells in early childhood. Scand J Immunol 5:721–726, 1976
Gathings WE, Lawton AR, Cooper MD: Immunofluorescent studies of the development of pre-B cells, B lymphocytes and immunoglobulin isotype diversity in humans. Eur J Immunol 7:804–810, 1977
Platts-Mills TA, De Gast GC, Webster ADB, Asherson GL, Wilkins SR: Two immunologically distinct forms of late onset hypogammaglobulinemia. Clin Exp Immunol 44:383–388, 1981
Pereira S, Webster ADB, Platts-Mills T: Immature B cells in fetal development and immunodeficiency: Studies of IgM, IgG, IgA and IgD production in vitro using Epstein-Barr virus activation. Eur J Immunol 12:540–546, 1982
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Fiorilli, M., Crescenzi, M., Carbonari, M. et al. Phenotypically immature IgG-bearing B cells in patients with hypogammaglobulinemia. J Clin Immunol 6, 21–25 (1986). https://doi.org/10.1007/BF00915360
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DOI: https://doi.org/10.1007/BF00915360