28
Î SS 94. Patients having hypogammaglobulinemia and thymoma should
be given a diagnosis of Good syndrome. (C)
Î SS 95. In patients with Good syndrome, thymomas should be excised. (C)
Selective IgA deficiency (SIGAD)
Î SS 96. Subjects older than 4 years with a serum IgA level <7 mg/dL
and normal serum IgG and IgM levels and in whom other causes
of hypogammaglobulinemia have been excluded should be given a
diagnosis of SIGAD. (C)
Î SS 97. Patients with serum IgA levels of less than the normal range for
age but >7 mg/dL should not be given a diagnosis of IgA deficiency. (C)
Î SS 98. Patients with SIGAD should be monitored over time for the
occurrence of complications. (C)
Î SS 99. Medication use should be investigated in patients with IgA
deficiency. (C)
Î SS 100. Aggressive antimicrobial therapy, prophylaxis, or both
should be used in patients with SIGAD and recurrent sinopulmonary
infections. (C)
Î SS 101. Atopic disease should be treated aggressively in patients with
SIGAD. (C)
Î SS 102. Rare patients with SIGAD might benefit from IVIG replacement
therapy. (C)
IgG subclass deficiency (IGGSD)
Î SS 103. A diagnosis of IGGSD should be considered for a patient
with recurrent infections, ≥1 IgG subclass levels less than the fifth
percentile, and normal total concentrations of IgG, IgM, and IgA. (C)
Î SS 104. The principles of management of IGGSD should follow those
presented for SIGAD and SAD. (C)
Specific antibody deficiency (SAD)
Î SS 105. The diagnosis of SAD should be given to patients older
than 2 years with recurrent respiratory tract infections, normal
immunoglobulin and IgG subclass levels, and impaired response to
pneumococcal capsular polysaccharide. (C)
Î SS 106. Patients with SAD might benefit from additional immunization
with conjugate pneumococcal vaccines, intensified use of antibiotics,
and in some cases a period of IgG replacement therapy. (C)
Predominantly Antibody Deficiencies