A 7 year old boy is seen for recurrent skin abscesses and repeated episodes of pneumonia. His parents say the abscesses are not very red or tender. He has chronic eczema and coarse facies. Laboratory studies show eosinophilia and markedly elevated IgE. Which of the following defects is most likely responsible?
- A)Defective Th17 cell differentiationGABARITO
- B)Deficient C5 through C9 membrane attack complex formation
- C)Absent B cell maturation in bone marrow
- D)Failure of neutrophils to adhere to endothelium
- E)Defective phagolysosome fusion
Explicação
Hyper IgE syndrome, also called Job syndrome, results from impaired Th17 differentiation, often due to STAT3 pathway abnormalities. Patients develop recurrent cold staphylococcal abscesses, eczema, elevated IgE, and recurrent pneumonias. Ver explicação completa e trilha adaptativa →