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Increased IgE; Decreased IgG, IgM
2%
10/485
Decreased IgE, IgM, IgA, IgG
6%
28/485
Increased IgE, IgA; Decreased IgM
3%
16/485
Increased IgE
9/485
Increased IgM; Decreased IgG, IgA, IgE
85%
413/485
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Increased IgM and decreased IgG, IgA, and IgE is characteristic of hyper-IgM syndrome, which results in severe pyogenic infections. Hyper-IgM syndrome is an immunodeficiency characterized by B and T cell dysfunction. It is most commonly caused by defective CD40L on helper T cells which prevents class switching. It often presents with severe pyogenic infections and recurrent sinopulmonary infections early in life. It should be noted though that this is a non-specific finding that is associated with numerous other causes such as viral infection. Immunoglobulin levels reveal increased IgM and decreased IgG, IgA, and IgE. The treatment is administration of IVIG. Incorrect Answers: Answer 1: Increased: IgE, and Decreased: IgG, IgM is not observed in hyper-IgM syndrome syndrome and is not characteristic of a specific immunodeficiency. Answer 2: Decreased: IgE, IgM, IgA, IgG is characteristic of X-linked (Bruton's) agammaglobulinemia. Answer 3: Increased: IgE, IgA, and decreased IgM is characteristic of Wiskott-Aldrich syndrome. Answer 4: Increased IgE is characteristic of hyper-IgE syndrome in which Th1 cells fail to produce interferon-gamma.
4.2
(5)
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