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231224s2015 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2015.08.017
|2 doi
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|a pubmed24n0841.xml
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|a (NLM)26342539
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|a (PII)S1521-6616(15)30033-4
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|a DE-627
|b ger
|c DE-627
|e rakwb
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| 041 |
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|a eng
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| 100 |
1 |
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|a Mensen, Angela
|e verfasserin
|4 aut
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| 245 |
1 |
0 |
|a Altered B-cell subsets and functional B-cell defects in selective IgM deficiency
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| 264 |
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|c 2015
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| 336 |
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|a Text
|b txt
|2 rdacontent
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| 337 |
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|a ƒaComputermedien
|b c
|2 rdamedia
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| 338 |
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|a ƒa Online-Ressource
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|2 rdacarrier
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| 500 |
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|a Date Completed 29.02.2016
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|a Date Revised 23.11.2015
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Primary selective IgM deficiency (sIgM) is characterized by diminished serum IgM, infections and autoimmunity. Although there is some evidence of B-cell defects the pathogenesis of sIgM is poorly understood. We determined peripheral B-cell subsets and IgM-expression levels in 31 adult sIgM patients by flow cytometry. In a subset of patients B-cell subset alterations and antibody-secreting cells were determined by flow cytometry and ELISpot assay after in vitro differentiation.Patients had significantly increased transitional, decreased IgM only, switched and non-switched memory B cells and decreased membrane IgM-expression levels on memory B-cell subsets compared to healthy controls. A strongly diminished B-cell differentiation and expansion capacity was observed in 5/6 investigated patients. Severely reduced IgM-secreting capacity was detected in 2/6 patients.Taken together, our results show altered B-cell subsets and severe functional B-cell defects in sIgM. This may provide a diagnostic tool and basis for subclassification of patients to study the pathogenetic background
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|a Journal Article
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|a Antibody-secreting cells
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4 |
|a ELISpot assay
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| 650 |
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4 |
|a Memory B cells
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| 650 |
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4 |
|a Primary immunodeficiency
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| 650 |
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4 |
|a Selective IgM deficiency
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| 650 |
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4 |
|a Transitional B cells
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| 650 |
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7 |
|a Antibodies
|2 NLM
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| 650 |
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|a Immunoglobulin M
|2 NLM
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| 700 |
1 |
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|a Krause, Torben
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Hanitsch, Leif Gunnar
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Meisel, Christian
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Kleint, Maximilian E
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Volk, Hans-Dieter
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Na, Il-Kang
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Scheibenbogen, Carmen
|e verfasserin
|4 aut
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| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 161(2015), 2 vom: 08. Dez., Seite 96-102
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
1 |
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|g volume:161
|g year:2015
|g number:2
|g day:08
|g month:12
|g pages:96-102
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| 856 |
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|u http://dx.doi.org/10.1016/j.clim.2015.08.017
|3 Volltext
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