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URN: urn:nbn:de:bsz:25-opus-6548
URL: http://www.freidok.uni-freiburg.de/volltexte/654/


Hornung, Marcus von

Molecular defects in B lymphocytes from patients with CVID- syndrome

Molekulare Defekte in B-Lymphozyten aus Patienten mit Antikörpermangelsyndrom

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Kurzfassung in Deutsch




Kurzfassung in Englisch

Common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease, the hallmark of which is hypogammaglobulinemia. A number of defects of T cell function and deficits in the memory B cell pool have been identified, but the underlying cause for this defect remains unknown. In vitro stimulation experiments with CVID peripheral blood B cells (CD19+) showed, that CVID B cells could secrete IgM, but no or only few IgG. Transplantation of human peripheral blood lymphocytes of two CVID patients into the spleen of immunodeficient mice showed that the functional B cell integrity was restored in one patient after 21d, (as measured in terms of IgG production), whereas in the second patient the serum antibody concentrations were only low. Analyses of CVID lymph nodes by immunohistology and FACS showed in three CVID patients numerous enlarged follicles with mostly well developed mantle zones. The follicles contained high percentages of activated and proliferating germinal center B cells, but plasma cells were absent. Analyses of CD27 showed that the percentage of memory B cells was strongly decreased, but whether they were generated within the lymph node tissues or whether they represent recirculating memory B cells has still to be determined. FACS analysis of CD21 expression showed in 2 CVID lymph nodes a high percentage of activated B cells (CD27+) with a disturbed CD21 surface expression. One CVID patient developed a Granulomatous Disease, which may cause or enhance the immunological abnormalities. Analysis of the lymph node from a patient with non-Hodgkin lymphoma showed that the structural integrity of the lymph node was totally lost. The important role of CD40 signalling for B cell activation and survival was investigated by activation of CVID naive B cells with human CD40L and showed that activation and survival was not altered, as measured by the expression of CD86 after more than 72 hours.


SWD-Schlagwörter: Lymphknoten , B-Lymphozyt , primärer Immundefekt , Keimzentrum
Freie Schlagwörter (englisch): B lymphocyte , primary immunodeficiency , germinal center
Institut: Fakultät für Biologie Dekanat
Fakultät: Fakultät für Biologie (bis Sept. 2002)
DDC-Sachgruppe: Biowissenschaften, Biologie
Dokumentart: Dissertation
Erstgutachter: Eibel, Hermann (PD Dr.)
Sprache: Englisch
Tag der mündlichen Prüfung: 10.02.2003
Erstellungsjahr: 2002
Publikationsdatum: 19.02.2003
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