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Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy

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Abstract

Marked polyclonal immunoglobulin (Ig)G4 hypergammaglobulinemia has exceptionally been reported. Here we report on two Algerian patients who presented a syndrome characterized by anemia, plasmacytic lymphadenopathy, renal manifestations, and a marked polyclonal IgG4 hypergammaglobulinemia leading to a hyperviscosity syndrome in one case. The IgG4-expressing cell percentage was significantly increased in the peripheral blood lymphocytes collected from the two patients upon diagnosis. Moreover, in contrast with normal sera, both patients’ sera significantly increased the percentage of IgG4-expressing cells when incubated with CD40-stimulated normal B lymphocytes. Similar effects were obtained with the culture supernatants of the patients’ activated T cells. Anti-interleukin (IL) 4 and/or anti-IL-13 antibodies were unable to antagonize the IgG4 production. IL-4 and IL-13 serum concentrations were found to be normal in the two patients. The increased IgG4 production was found to be mediated by soluble factor(s), most probably secreted by activated T cells, which did not require the signal transducer and activator of transcription 6 signaling pathway.

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Acknowledgements

This work was supported in part by grants from the Fondation de France and Cent pour Sang la Vie. E.B. was the recipient of a fellowship from Sidaction Ensemble contre le Sida. The authors thank Drs. M.J. Terrier-Lacombe, M.C. Gubler and M. Sibony for providing histological samples collected from patients, Dr. D. Bengoufa for autoantibodies detection, and Dr. F. Agbalika for virological analyses. The experiments comply with the current laws of the country in which they were performed inclusive of ethics approval.

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Authors and Affiliations

  1. Department of Clinical Immunology, Hôpital Saint-Louis, AP-HP, Paris, France

    Emmanuelle Boulanger & Jean-Pierre Clauvel

  2. Inserm E0351, Department of Immunology, Hôpital Nord, Amiens, France

    Vincent Fuentes, Valérie Gouilleux-Gruart & Kaiss Lassoued

  3. Laboratory of Pathology, Hôpital Saint-Louis, AP-HP, Paris, France

    Véronique Meignin

  4. Laboratory of Pathology, Hôpital Tenon, AP-HP, Paris, France

    Béatrice Mougenot

  5. IFR105, Laboratory of Immunopathology, Hôpital Saint-Louis, Paris, France

    Sylvaine Labaume

  6. UMR CNRS 6101, Laboratory of Immunology, CHU, Limoges, France

    Michel Cogné

  7. Laboratory of Immunochemistry, Hôpital Tenon, AP-HP, Paris, France

    Pierre Aucouturier

  8. Department of Nephrology, Hôpital Tenon, AP-HP, Paris, France

    Pierre Ronco

  9. Service d’Immunopathologie Clinique, Hôpital Saint-Louis, 1 avenue Claude Vellefaux, 75 475, Paris Cedex 10, France

    Emmanuelle Boulanger

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  1. Emmanuelle Boulanger
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  2. Vincent Fuentes
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  3. Véronique Meignin
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  4. Béatrice Mougenot
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  7. Michel Cogné
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  8. Pierre Aucouturier
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  9. Jean-Pierre Clauvel
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  10. Pierre Ronco
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  11. Kaiss Lassoued
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Correspondence to Emmanuelle Boulanger.

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Boulanger, E., Fuentes, V., Meignin, V. et al. Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy. Ann Hematol 85, 833–840 (2006). https://doi.org/10.1007/s00277-006-0158-5

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  • DOI: https://doi.org/10.1007/s00277-006-0158-5

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