1. Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy
- Author
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Pierre Ronco, Pierre Aucouturier, Vincent Fuentes, Michel Cogné, Kaiss Lassoued, Valérie Gouilleux-Gruart, Véronique Meignin, Béatrice Mougenot, Jean-Pierre Clauvel, Sylvaine Labaume, Emmanuelle Boulanger, Gvh et Gvl : Physiopathologie Chez l'Homme et Chez l'Animal, Incidence et Role Therapeutique, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pathologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'anatomie pathologique [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Développement normal et pathologique des lymphocytes et signalisation, Université de Picardie Jules Verne (UPJV)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Physiologie Moléculaire de la Réponse Immune et des Lymphoproliférations (PMRIL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Université de Limoges (UNILIM), Maladies à prions et système immunitaire, IFR65-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
- Subjects
Male ,Pathology ,MESH: Anemia ,Gene Expression ,Lymphocyte Activation ,0302 clinical medicine ,Hypergammaglobulinemia ,Lymphocytes ,Cells, Cultured ,MESH: Immunoglobulin G ,0303 health sciences ,MESH: Plasma Cells ,Proteinuria ,Hematology ,MESH: Middle Aged ,biology ,MESH: Culture Media, Conditioned ,Anemia ,General Medicine ,Middle Aged ,3. Good health ,MESH: STAT6 Transcription Factor ,030220 oncology & carcinogenesis ,[SDV.IMM]Life Sciences [q-bio]/Immunology ,Female ,Kidney Diseases ,medicine.symptom ,Antibody ,MESH: Cells, Cultured ,medicine.medical_specialty ,MESH: Gene Expression ,Adolescent ,Plasma Cells ,Nephropathy ,03 medical and health sciences ,MESH: Lymphatic Diseases ,MESH: Hypergammaglobulinemia ,Internal medicine ,parasitic diseases ,Hyperviscosity syndrome ,medicine ,Humans ,MESH: Lymphocyte Activation ,Lymphatic Diseases ,030304 developmental biology ,MESH: Adolescent ,MESH: Kidney Diseases ,MESH: Humans ,business.industry ,medicine.disease ,MESH: Male ,Polyclonal antibodies ,Culture Media, Conditioned ,Immunoglobulin G ,Immunology ,biology.protein ,MESH: Lymphocytes ,business ,STAT6 Transcription Factor ,MESH: Female - Abstract
International audience; 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