Patients with Wiskott-Aldrich syndrome show severely impaired responses to vaccines such as the tetanus vaccine, which is composed of the inactivated tetanus toxin (i.e., tetanus toxoid). Yet, these patients can generate normal antibody responses to bacterial polysaccharide antigens. This selective defect in antibody responses in Wiskott-Aldrich syndrome patients is due to:
A) Defective antigen presentation by dendritic cells
B) Impaired phagocytic function of macrophages and dendritic cells
C) Defective polarized secretion of cytokines by CD4 T cells
D) Impaired B cell activation to monovalent, rather than polyvalent antigens
E) Impaired migration of B cells to germinal centers

Question

Quizplus · Accepted Answer

DWiskott-Aldrich syndrome primarily affects the ability of immune cells to respond to certain types of antigens. The syndrome leads to a defect in the ability of CD4 T cells to properly secrete cytokines in a polarized manner (C), which is crucial for the activation of B cells in response to protein antigens like the tetanus toxoid. Additionally, patients have an impaired B cell activation response to monovalent antigens, which are antigens with a single epitope, as opposed to polyvalent antigens, such as bacterial polysaccharides, which have multiple epitopes and can directly activate B cells without T cell help (D).

Patients with Wiskott-Aldrich Syndrome Show Severely Impaired Responses to Vaccines