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Predominantly Antibody Deficiencies
Agammaglobulinemia
Î SS 81. Patients with very low or undetectable serum immunoglobulin
concentrations and very low or undetectable circulating B lymphocytes
with normal T-cell numbers and function should be given a diagnosis
of agammaglobulinemia. (C)
Î SS 82. Agammaglobulinemia should be managed aggressively with
antimicrobials, IgG replacement, and careful attention to pulmonary
status. (C)
Î SS 83. Enteroviral meningoencephalitis in patients with
agammaglobulinemia should be treated with high doses of IVIG with
measurable antibody to the infecting virus. (C)
Î SS 84. Lung transplantation should be considered for patients with
agammaglobulinemia and life-threatening chronic lung disease. (C)
Common variable immunodeficiency (CVID)
Î SS 85. The diagnosis of CVID should be considered in male or female
subjects older than 4 years who have low IgG and IgA levels and
impaired antibody response but do not have genetic lesions or other
causes of primary or secondary antibody deficiency. (C)
Î SS 86. Grouping of patients with CVID based on analysis of B-cell
subsets in the periphery might be useful and should be considered. (C)
Î SS 87. Selected diseases, molecular defects, or both should be
considered in patients presenting with symptoms and signs consistent
with CVID. (C)
Î SS 88. CVID should be managed aggressively with antimicrobials,
IgG replacement, and careful attention to pulmonary status. (C)
Î SS 89. Gastrointestinal status should be monitored regularly in
patients with CVID. (C)
Î SS 90. Vigilance for possible autoimmune diseases should be
maintained during follow-up of patients with CVID. (C)
Î SS 91. Vigilance for nonmalignant and malignant lymphoproliferative
disease should be maintained during follow-up of patients with CVID. (C)
Î SS 92. Autoimmune, lymphoproliferative, or malignant diseases
associated with CVID are treated as they would be in other clinical
settings. (C)
Î SS 93. Stem cell transplantation can be considered for patients with
CVID with malignancy or severe organ damage. (C)
Predominantly Antibody Deficiencies
