Sigune Goldacker

TL;DR: The clinical evaluation of 303 patients with the established diagnosis of CVID demonstrated a significant coincidence of granulomatous disease, autoimmune cytopenia, and splenomegaly, and an improved classification for CVID (EUROclass), separating patients with nearly absent B cells, and expansion of transitional or CD21(low) B cells.

TL;DR: It is suggested that CD21low B cells represent a human innate-like B cell population characterized by the low expression of CD21, which has close resemblance to the gene expression profile, phenotype, function and preferential tissue homing of murine B1 B cells.

TL;DR: HSCT in patients with CVID was beneficial in most surviving patients; however, there was a high mortality associated with the procedure, so this therapeutic approach should only be considered in carefully selected patients in whom there has been extensive characterization of the immunologic and/or genetic defect underlying the CVID diagnosis.

TL;DR: It is shown that deficiency of adenosine deaminase 2 (DADA2) in patients with antibody deficiency and the clinical picture of the disease in adulthood in adulthood is similar to that of absence of DADA2.

TL;DR: Identification of excessive IFN‐&ggr; production by blood and lymph node–derived T cells of patients with CVID with immune dysregulation will offer new therapeutic avenues for this subgroup.