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Defined Blocks in Terminal Plasma Cell Differentiation of Common Variable Immunodeficiency Patients¹

Nadine Taubenheim^*, Marcus von Hornung^*, Anne Durandy, Klaus Warnatz, Lynn Corcoran, Hans-Hartmut Peter and Hermann Eibel^2,*

^* Clinical Research Unit for Rheumatology, University Hospital of Freiburg, Freiburg, Germany; Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 429, Hôpital Necker-Enfants Malades, Paris, France; Division of Rheumatology and Clinical Immunology, Department of Medicine, University Hospital of Freiburg, Freiburg, Germany; The Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia

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.

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