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.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Boulanger E,Fuentes V,Meignin V,Mougenot B,Labaume S,Gouilleux-Gruart V,Cogné M,Aucouturier P,Clauvel JP,Ronco P,Lassoued Kdoi
10.1007/s00277-006-0158-5subject
Has Abstractpub_date
2006-12-01 00:00:00pages
833-40issue
12eissn
0939-5555issn
1432-0584journal_volume
85pub_type
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