is an extremely rare disease affecting only males . The disease
is thought to affect less than 1 child in a million . The disease
can manifest in many forms. The three most common are fatal infectious
mononucleosis (FIM), dysgammaglobunemia, and malignant lymphoma.
Less common problems may include aplastic anemia and vasculitis.
The average age of onset is 2.5 years of age and nearly 75% of the
children are deceased before 10 years of age. The oldest known survivor
was 44 years of age. Exposure to EBV(mononucleosis) is not required
for disease forms other than FIM.
EBV is a widespread herpes virus, more common than the chicken pox.
It affects about 90% of our population by adulthood. EBV infection
in infants and children is usually asymptomatic, but in adolescents
and adults glandular fever is often seen. In males with XLP, an
uncontrolled increase in the number of T and B cells in response
to mono often leads to death from liver or bone marrow failure.
Children with XLP who survive EBV can later develop lymphomas or
other forms of this disease. The only definitive treatment currently
available is allogenic (donor) bone marrow transplants. IgG therapy
will not protect against EBV.