The number of circulating CD4+CD25highFoxp3+ T lymphocytes is transiently elevated in the early childhood of transient hypogammaglobulinemia of infancy patients

Transient hypogammaglobulinemia of infancy

“…Because TGFβ signaling is required for the generation of T regulatory cells it was speculated that mutations in the TGFβ gene may explain this finding. Unfortunately, mutation analysis of this gene did not support this assumption (Rutkowska et al 2011(Rutkowska et al , 2013.…”

“…Rutkowska et al (2011Rutkowska et al ( , 2013 demonstrated that THI patients, unlike CVID patients, had increased levels of T regulatory cells and that may be used to differentiate between the 2 groups. Nonetheless, it is still not used as routine laboratory test.…”

Phenotypic Parameters Predict Time to Normalization in Infants with Hypogammaglobulinemia

“…At the time of initial evaluation, immunologists may use aggregate immunoglobulin levels or initial IgG levels to predict probability of resolution, although the aggregate immunoglobulin level does so with greater power. Although other markers such as cytokine measurements [3] or regulatory T-cell measurements, [5] have been associated with resolution of hypogammaglobulinemia, these markers are not readily available outside of a research setting and are therefore of little use to most clinicians. Aggregate immunoglobulin as a percentage of minimal normal and IgG as a percentage of minimal normal can be easily calculated by a practicing clinician and may be of significant practical utility.…”

“…[3] A report in 1981 associated THI with numerical and functional deficiency of helper T-cells [4]; more recently, however, THI has been associated with a transient increase in numbers of regulatory T-cells. [5] Complicating the assessment and prognostication about infants with hypogammaglobulinemia is the reality that a diagnosis of THI can only be established in retrospect, after immunoglobulin levels have normalized. [6] Without question, some Infants with hypogammaglobulinemia will not have resolution of their deficiency and those infants with persistent hypogammaglobulinemia may be at increased risk for recurrent infection and autoimmune disease.…”

Transient Hypogammaglobulinemia of Infancy

“…12 Rutkowska et al have suggested that IVIG may increase the suppressive action of Treg cells and thus prolong hypogammaglobulinemia, but no supportive evidence was presented. 21 As an alternative to immune globulin replacement, some reports have recommended antimicrobial prophylaxis, but this therapy also has been recommended only for patients with frequent or severe infections. 9,15,19,24 …”