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Renner, Ellen D.; Kraetz, Carolin E.; Orange, Jordan S.; Hagl, Beate; Rylaarsdam, Stacey; Notheis, Gundula; Durandy, Anne; Torgerson, Troy R. and Ochs, Hans D. (2021): Class Switch Recombination Defects: impact on B cell maturation and antibody responses. In: Clinical Immunology, Vol. 222, 108638

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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 IKK gamma 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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