PMC8727997_01
Absolute number (A, C) and percentage (B, D) of naive and unswitched- and switched-memory B cells in patient 1 and 2.
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Lymphocyte immunophenotyping showed a normal B-cell count (420 cells/microl) but fewer unswitched-memory B cells (1%; when normal = 4%-23%) and switched-memory B cells (0%; when normal = 5%-18%) and more naive B cells (99%; when normal = 60%-89%) ( Figure 1 ).
PMC8727997_02
Immunoglobulies levels during folow up for patient 1 (A) and patient 2 (B).
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IgG trough levels were approximately 6 g/L, with very low IgA and IgM, despite continued IRT (Privigen ) at 0.4 g/kg monthly (see  Figure 2 ). In February 2015, extended lymphocyte immunophenotyping showed normal pan B-cell levels but lower levels of switched- and unswitched-memory B cells. IgG trough levels at 5 g/L. In July 2020, after a 10-week IRT interruption, IgG levels were at 6 g/L. In all previous years, IgA had been undetectable, while IgM was normal (see  Figure 2 ).