to confirm the physician’s suspicion of a congenital immunodeficiency. A frameshift
mutation in the perforin gene PRF1 was found on both chromosomes. Kristen was
diagnosed with the rare, potentially life-threatening disease known as familial
hemophagocytic lymphohistiocytosis (FHL). Cytotoxic and aggressive
immunosuppressive chemotherapy was administered followed by a matched unrelated
hematopoietic stem cell transplant. Two years later Kristen is a healthy toddler. Which of
the following would not be consistent with the etiology of FHL?
a.impaired cytotoxic activity of CD8 T cells
b.inhibition of transcriptional activators required for IL-2 synthesis
c.inability to kill virus-infected cells
d.persistent activation of CD8 T cells causing secretion of large amounts of IFN-
e.IFN- activation of macrophages which in turn drives the production of IL-6, TNF- ,
and other pro-inflammatory molecules.
Macrophages bear on their surface receptors for all of the following except _____. (Select
all that apply).
a.mannose
b.glucans
c.C3b
d.muramyl dipeptide
e.lipopolysaccharide
f.lipoteichoic acid
g.CpG-rich bacterial DNA.