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Media type:
E-Article
Title:
Defined Blocks in Terminal Plasma Cell Differentiation of Common Variable Immunodeficiency Patients
Contributor:
Taubenheim, Nadine;
von Hornung, Marcus;
Durandy, Anne;
Warnatz, Klaus;
Corcoran, Lynn;
Peter, Hans-Hartmut;
Eibel, Hermann
imprint:
The American Association of Immunologists, 2005
Published in:The Journal of Immunology
Language:
English
DOI:
10.4049/jimmunol.175.8.5498
ISSN:
0022-1767;
1550-6606
Origination:
Footnote:
Description:
<jats:title>Abstract</jats:title>
<jats:p>Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by defective Ab production and recurrent bacterial infections. The largely unknown causes are likely to comprise a diverse set of genetic or acquired defects. In this study, we investigated terminal B cell differentiation in lymph nodes from CVID patients. Up to the germinal center B cell stage, B cell differentiation was normal but terminal plasma cell development was found to be impaired. Using differential Blimp-1 and Syndecan-1 expression in controls, we defined three different plasma cell subsets that correspond to progressive developmental stages locating to different sites in the lymph node. In the CVID patients, we could only detect one or two of these subsets indicating a defective differentiation. Thus, terminal plasma cell differentiation was found to be impaired despite normal expression of Blimp-1. B cells reaching only the first stage of plasma cell differentiation were further unable to undergo isotype switching and to up-regulate activation markers on B cells stimulated in vitro.</jats:p>