Reduced memory B cells in patients with hyper IgE syndrome.
Dominant-negative mutations in STAT-3 have recently been found in the majority of patients with sporadic or autosomal-dominant hyper IgE syndrome (HIES). Since STAT-3 plays a role in B cell development and differentiation, we analyzed memory B cells in 20 patients with HIES, 17 of which had STAT-3 mutations. All but four patients had reduced non-switched and/or class-switched memory B cells. No reduction in these B cell populations was found in 16 atopic dermatitis patients with IgE levels above 1000 KU/L. There was no correlation between the reduction of memory B cells and the ability to produce specific antibodies. Moreover, there was no correlation between the percentage of memory B cells and the infection history. Analysis of memory B cells can be useful in distinguishing patients with suspected HIES from patients with atopic disease, but probably fails to identify patients who are at high risk of infection.
Speckmann
C
C
Center for Pediatrics and Adolescent Medicine, University of Freiburg, Mathildenstr 1, 79106 Freiburg, Germany.
Enders
A
A
Woellner
C
C
Thiel
D
D
Rensing-Ehl
A
A
Schlesier
M
M
Rohr
J
J
Jakob
T
T
Oswald
E
E
Kopp
M V
MV
Sanal
O
O
Litzman
J
J
Plebani
A
A
Pietrogrande
M C
MC
Franco
J L
JL
Espanol
T
T
Grimbacher
B
B
Ehl
S
S
D000293:Adolescent; D000328:Adult; D000914:Antibodies, Viral / Q000097:blood; D000917:Antibody Formation; D001402:B-Lymphocytes / Q000276:immunology* / Q000473:pathology; D002648:Child; D015331:Cohort Studies; D004247:DNA / Q000737:chemistry / Q000235:genetics; D003876:Dermatitis, Atopic / Q000235:genetics / Q000276:immunology / Q000473:pathology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005434:Flow Cytometry; D005838:Genotype; D006801:Humans; D007073:Immunoglobulin E / Q000276:immunology / Q000378:metabolism; D007156:Immunologic Memory / Q000235:genetics / Q000276:immunology*; D007589:Job Syndrome / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009154:Mutation; D050796:STAT3 Transcription Factor / Q000235:genetics / Q000276:immunology; D055815:Young Adult
D000914:Antibodies, Viral; D050796:STAT3 Transcription Factor; D007073:Immunoglobulin E; D004247:DNA