Review Article

Pediatric Selective IgM Immunodeficiency

Table 3

Immunologic abnormalities in SIgMID.

T cell functions Increased IgM-specific T cell suppressor function [35, 40, 41]; normal T cell suppression function [4244]
Excessive isotype nonspecific T cell suppressive activity [40]; increased IgM specific suppressive T cell function [35]
Defect in T cell help [10, 26]; normal T cell help [17, 42, 44, 45]
Nonspecific T cell abnormalities [26]; normal T cell function [42]

B cell function Defect in B cell differentiation into IgM-immunoglobulin secreting cells [40, 46, 47]

T and B cell enumeration and phenotype Normal peripheral T and B cell phenotypes [42, 43, 47, 48]; increased CD8+ cells and inverted CD4/CD8 ratios [40]; increased CD4+ cells and decreased CD8+ cells [47]
Reduced number of IgM secreting B cells [45, 46] with a failure of secreted mu mRNA synthesis [46]; normal surface IgM expression on B cells [10, 17, 35, 43, 4749]; normal secreted mu mRNA synthesis [49]

Mitogen/antigen stimulation Mitogen and antigen stimulated B cell proliferation assays with normal IgM responses [11, 42]; decreased antigen proliferation IgM responses [4, 17, 40, 41, 4549]
Deficient IgM responses to viral antigens and/or endotoxin containing vaccines and deficient isohemagglutinin antibodies [24]
Failure to respond to antigen challenge with tetanus toxoid, pneumococcal vaccine, meningococcus vaccine, Salmonella O and H antigens, and typhus-paratyphus vaccine [1012, 19, 42, 50]

Complement No complement deficits [10]

Delayed hypersensitivity Reduced delayed cutaneous hypersensitivity [10, 17]; normal delayed cutaneous hypersensitivity [11, 42]

Phagocytosis Normal phagocytosis and killing of encapsulated bacteria [10]; nominally affected opsonification of yeast particles [11]; select opsonic defect against Pseudomonas [51]