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Renner, E.D. ; Krätz, C.E. ; Orange, J.S.* ; Hagl, B. ; Rylaarsdam, S.* ; Notheis, G. ; Durandy, A.* ; Torgerson, T.R.* ; Ochs, H.D.*

Class switch recombination defects: Impact on B cell maturation and antibody responses.

Clin. Immunol. 222:108638 (2021)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
To assess how B cell phenotype analysis correlates with antigen responses in patients with class switch recombination defects (CSRD) we quantified memory B cells by flow-cytometry and immunized CSRD patients with the neoantigen bacteriophage phiX174 (phage). CSRD patients showed uniformly absent or markedly reduced switched memory B cells (IgM−IgD−CD27+). CD40L patients had reduced CD27+ memory B cells (both non-switched and switched). In NEMO patients, results varied depending on the IKKγ gene variant. Three of four AID patients had normal percentages of CD27+ memory B cells while CD27+IgM−IgD− switched memory B cells were markedly reduced in all AID patients. Antibody response to phage was remarkably decreased with lack of memory amplification and class-switching in immunized CD40L, UNG deficient, and NEMO patients. Distinct B-cell phenotype pattern correlated with abnormal antibody responses to a T-cell dependent neoantigen, representing a powerful tool to identify CSRD patients.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords B Cell Development ; Class Switch Recombination Defect (csrd) ; Clinical Immunology ; Hyper-igm Syndromes (higm) ; Immunodeficiencies; Hyper-igm Syndrome; Anhidrotic Ectodermal Dysplasia; Induced Cytidine Deaminase; Cd40 Ligand; Primary Immunodeficiency; Deficiency; Mutations; Activation; Gene
ISSN (print) / ISBN 1521-6616
e-ISSN 1521-7035
Journal Clinical Immunology
Quellenangaben Volume: 222, Issue: , Pages: , Article Number: 108638 Supplement: ,
Publisher Elsevier
Publishing Place 525 B St, Ste 1900, San Diego, Ca 92101-4495 Usa
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Environmental Medicine (IEM)
Grants Jeffrey Modell Foundation
Helmholtz-Gemeinschaft Future Topic "Immunology and Inflammation"
CKCARE foundation
Wilhelm-Sander foundation
German Research Foundation