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Information

Deficiencies that affect B cells are the most common primary immunodeficiencies and account for ~60-70% of all cases. Defective antibody production predisposes to invasive pyogenic bacterial infections as well as recurrent sinus and pulmonary infections. Complete lack of antibody production (agammaglobulinemia) predisposes to disseminated enteroviral infections causing meningoencephalitis, hepatitis, and a dermatomyositis-like disease. Diagnosis relies on the determination of serum Ig level.

Treatment: B Cell/Immunoglobulin Deficiency Syndromes

IV immunoglobulin administration (only for pts who have recurrent bacterial infections and are deficient in IgG):

  • Starting dose 400-500 mg/kg given every 3-4 weeks
  • Adjust dose to keep trough IgG level 800 mg/dL
  • SC administration usually once a week can be considered in selected pts.

Outline

Section 12. Allergy, Clinical Immunology, and Rheumatology