Why was the number of CD8 T cells in Tatiana and

Alexander decreased despite normal levels of CD4 T cells?

The intrathymic maturation of CD8 T cells depends on the
expression of MHC class I molecules on thymic epithelial
cells. Conversely, maturation as CD4 T cells requires
interactions with the MHC class II molecules also present
on thymic epithelium. Because Tatiana and Alexander do
not lack MHC class II molecules, their CD4 T cells are
normal and they have normal humoral immunity.
When the T-cell antigen receptors on their CD8 T cells
were examined, it turned out that they were
all : receptors and none were : receptors. How do you
explain this?
The maturation of CD8 T cells bearing : chains occurs
after these cells emigrate from the thymus and is
independent of MHC class I expression, whereas the
maturation of : CD8 T cells occurs in the thymus and is
dependent on the expression of MHC class I molecules.
Tatiana and Alexander had normal delayed-type
hypersensitivity responses to tuberculin and Candida. Is
this surprising in view of their deficiency in CD8 T cells?
No. Delayed-type hypersensitivity reactions are provoked
by antigen-specific CD4 T cells (see Case 8).
Why did Tatiana and Alexander have high levels of serum
IgG?
The factors that help B cells to mature and secrete
immunoglobulins are derived from activated CD4 T cells.
These cells were normal in Tatiana and Alexander. Factors
that suppress B-cell responses are secreted by CD8 T
cells, of which the children had very few. They were
therefore not very efficient at terminating B cell-mediated
humoral immune reactions and tended to overproduce
antibody. In Case 8, we saw the opposite phenomenon in
patients with MHC class II deficiency, who have a

deficiency of CD4 T cells and very low levels of serum

immunoglobulins.
Genetic defects have also been found in the genes
encoding TAP1 and TAPBP. Do you think that the clinical
course in these patients would differ from that observed in
Tatiana and Alexander?
The patients with TAP1 deficiency clinically resembled
those with TAP2 deficiency.