Immunology

4. Assume you are a resident at a renowned pediatric hospital. A patient
is referred to you for evaluation. He is a 5 year-old male that has just
recovered from bacterial meningitis following otitis media (an ear
infection). His records reveal a history of bacterial ear and upper
respiratory tract infections beginning at age 1, and he has been
hospitalized four times for complications resulting from these
illnesses. In contrast, he recovered normally from both measles and
chickenpox. A family history reveals the child had three siblings: a
healthy older brother and sister, as well as a brother that died in
infancy of acute Streptococcal meningitis. His mother mentions to you
that both of her brothers (the boy’s maternal uncles) died of severe
childhood pneumonia. You have a series of tests done to assess the
patient. These tests and results are shown below:

TEST RESULT

Circulating IgM levels undetectable (<1% of normal values)

Circulating IgG levels undetectable (< 1% of normal values)

Lymphocyte count normal

Circulating surface IgM+ cells normal

Circulating surface IgG+ cells undetectable (<1% of normal values)

Circulating CD3+CD4+ cells normal

Circulating CD3+CD8+ cells normal

MLR to allogeneic (HLA disparate) cells normal

CML to allogeneic (HLA disparate cells normal

 

Based on this information:

a): Specify the child’s problem in immunologic terms, and tell how it
explains his symptoms. 

b). Speculate BRIEFLY on the underlying mechanism of the child’s
disease, AND its likely mode of transmission. 

c). What would you do to treat this child? What potential problems do
you foresee with your treatment?