| HUMORAL AND CELL-MEDIATED DEFICIENCIES | ||
| DISEASE | IMMUNE-SYSTEM DEFICIENCY | POSSIBLE MECHANISM |
| HUMORAL DEFICIENCIES | ||
| X-linked agammaglobulinemia (XLA) |
Reduction in B-cell count Absence of immunoglobulins |
Block in maturation of pre-B cells due to defective V-D-J gene arrangement |
| X-linked hyper-IgM (XHM) syndrome |
Low levels of IgG and IgA Very high levels of IgM |
Defect in class switching |
| Common variable hypogammglobulinemia (CVH) |
Decreased plasma-cell levels but usually normal B-cell levels Variable reduction in secreted Ig of all isotopes |
Defective differentiation of B cells to plasma cells due to defective processing of Ig transcipts, lack of cytokine receptors on B-cells, or abnormal T-cell response |
| Selective immunoglobulin deficiencies |
Decreased levels of one or more Ig isotopes |
Defect in maturation of plasma cells or necessary T-cell-derived cytokines |
| CELL-MEDIATED DEFICIENCIES | ||
| DiGeorge syndrome | Decreased T-cell counts | Lack of T-cell maturation due to absence of thymus |
| Nude mice | Decreased T-cell counts | Lack of T-cell maturation due to absence of thymus |