Transient CD80 expression defect in a patient with variable immunodeficiency and cyclic neutropenia.
CD80 expression on stimulated B cells of 9 common variable immunodeficiency (CVID) patients and 10 healthy control individuals was examined. Here we report on an exceptional CVID patient with recurrent episodes of cyclic neutropenia, skin vasculitis and recurrent infections associated with a transient, but reproducible CD80 expression defect on stimulated B cells. Concomitantly serum Ig levels were markedly reduced and B cell counts were low. Interestingly, after recovery from an episode of neutropenia and vasculitis we observed an improvement of the CD80 expression as well as an increase in the number of B cells and a recovery of IgM and IgG production in vivo and in vitro.
Moser
C
C
Abt. Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik, Freiburg, Germany.
Schlesier
M
M
Dräger
R
R
Eibel
H
H
Peter
H H
HH
D001402:B-Lymphocytes / Q000276:immunology*; D018122:B7-1 Antigen / Q000096:biosynthesis*; D017074:Common Variable Immunodeficiency / Q000209:etiology*; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000172:deficiency; D008213:Lymphocyte Activation / Q000276:immunology; D018655:Lymphocyte Count; D008875:Middle Aged; D009503:Neutropenia / Q000209:etiology*; D010507:Periodicity; D012871:Skin Diseases / Q000209:etiology*; D014657:Vasculitis / Q000209:etiology*
D018122:B7-1 Antigen; D007136:Immunoglobulins