“…As predicted by our previous studies, we have confirmed the presence of germinal centres and isotype switched B lymphocytes in rHIM [12].…”
Section: Introductionsupporting
confidence: 91%
“…High-resolution CT scans of the thorax have not shown bronchiectasis. As previously noted, he has normal CD40 ligand expression (11.5 % CD3+ CD40L+cells on PMA/Calcium ionophore activation) [12] and normal sequence compared with a patient with XHIM [12,14,15]. He has wild type sequence for the UNG, CD40 and AICDA genes [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 83%
“…As previously noted, he has normal CD40 ligand expression (11.5 % CD3+ CD40L+cells on PMA/Calcium ionophore activation) [12] and normal sequence compared with a patient with XHIM [12,14,15]. He has wild type sequence for the UNG, CD40 and AICDA genes [12]. His lymphocytes were able to produce IgG4 in vitro without the need for exogenous CD40 ligand [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 85%
“…In contrast, his IgM levels have been as high as 16 g/l (0.4-2.5) but generally have remained at approximately 4-6 g/l. He was initially treated with intramuscular immunoglobulin but subsequently placed on high dose IVIG and then subcutaneous immunoglobulin [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 99%
“…Patients with rHIM may not be easily recognized. Review of the literature suggests (i) this is an extremely rare syndrome even prior to widespread vaccination of susceptible women [11], (ii) rHIM patients may have milder immune defects than those with XHIM [12], and (iii) their condition may improve over time [13,14].…”
In contrast to XHIM where germinal centres are absent, the presence of small numbers of relatively normal germinal centres explain our previous identification of isotype switched memory B cells in rHIM.
“…As predicted by our previous studies, we have confirmed the presence of germinal centres and isotype switched B lymphocytes in rHIM [12].…”
Section: Introductionsupporting
confidence: 91%
“…High-resolution CT scans of the thorax have not shown bronchiectasis. As previously noted, he has normal CD40 ligand expression (11.5 % CD3+ CD40L+cells on PMA/Calcium ionophore activation) [12] and normal sequence compared with a patient with XHIM [12,14,15]. He has wild type sequence for the UNG, CD40 and AICDA genes [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 83%
“…As previously noted, he has normal CD40 ligand expression (11.5 % CD3+ CD40L+cells on PMA/Calcium ionophore activation) [12] and normal sequence compared with a patient with XHIM [12,14,15]. He has wild type sequence for the UNG, CD40 and AICDA genes [12]. His lymphocytes were able to produce IgG4 in vitro without the need for exogenous CD40 ligand [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 85%
“…In contrast, his IgM levels have been as high as 16 g/l (0.4-2.5) but generally have remained at approximately 4-6 g/l. He was initially treated with intramuscular immunoglobulin but subsequently placed on high dose IVIG and then subcutaneous immunoglobulin [12].…”
Section: Patient History and Previous Findingsmentioning
confidence: 99%
“…Patients with rHIM may not be easily recognized. Review of the literature suggests (i) this is an extremely rare syndrome even prior to widespread vaccination of susceptible women [11], (ii) rHIM patients may have milder immune defects than those with XHIM [12], and (iii) their condition may improve over time [13,14].…”
In contrast to XHIM where germinal centres are absent, the presence of small numbers of relatively normal germinal centres explain our previous identification of isotype switched memory B cells in rHIM.
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