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