1. Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy.
- Author
-
Boulanger E, Fuentes V, Meignin V, Mougenot B, Labaume S, Gouilleux-Gruart V, Cogné M, Aucouturier P, Clauvel JP, Ronco P, and Lassoued K
- Subjects
- Adolescent, Cells, Cultured, Culture Media, Conditioned pharmacology, Female, Gene Expression drug effects, Humans, Immunoglobulin G metabolism, Kidney Diseases blood, Lymphocyte Activation, Lymphocytes metabolism, Male, Middle Aged, Plasma Cells pathology, STAT6 Transcription Factor blood, STAT6 Transcription Factor metabolism, Anemia complications, Hypergammaglobulinemia complications, Immunoglobulin G blood, Kidney Diseases complications, Lymphatic Diseases complications
- 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.
- Published
- 2006
- Full Text
- View/download PDF