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Abstracts (complete list) - Wissenschaft Online

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Baerbel Keller, Mirzokhid Rakhmanov, Sylvia Gutenberger, Sigune Goldacker, Dirk<br />

Holzinger, Elisabeth Nikolopoulus, Paul Fisch, Hermann Eibel, Hans-Hartmut Peter,<br />

Klaus Warnatz<br />

CD21low B Cells Represent a Polyclonally Activated B Cell<br />

Population Exposed to Type I Interferons<br />

Most patients with common variable immunodeficiency (CVID) exhibit a severely<br />

disturbed homeostasis of the circulating B cell pool. About 10% of these patients are<br />

characterized by the expansion of B cells with an unusually low expression of CD21.<br />

These CD21lowCD38low B cells exhibit an activated phenotype with increased cell size<br />

and elevated levels of CD86. Spectrotyping of sorted cells identified a polyclonal origin<br />

of CD21low B cells. A similar expansion of CD21low B cells in SLE as well as HIV<br />

infected patients suggested a potential role of type I interferon stimulation. Gene<br />

expression analysis for MxA, OAS1 and IFIT1 of FACS sorted CD21low B cells versus<br />

naive B cells of HD and CVID patients confirmed the interferon signature in CD21low B<br />

cells. Since HHV8 was suggested as a possible pathogen in a subgroup of CVID patients<br />

we tested our patients with expanded CD21low B cells and granulomatous disease, but<br />

could not confirm a role of HHV8 in our cohort. Currently we are investigating polyclonal<br />

activation of B cells via different Toll like receptors as a possible source of CD21low B<br />

cell differentiation.

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