Primary B Cell Immunodeficiency

Question 1

Transient Hypogammaglobulinemia of infancy

Answer 1

• Presents around 6 mo
• Recurrent pyogenic infections
• Tx: give gamma globulins
• Gets better with age

Question 2

Common Variable Hypogammaglobulinemia

Answer 2

• B cell differentiation block
• onset: any age
• tx: give gamma globulins
• May develop autoantibodies
• increased susceptibility to viral and fungal infections

Question 3

Selective IgA Deficiency

Answer 3

• Chronis sinus infections
• White people
• May have transfusion rxns (anti-Iga present)
• increased incidence AI diseases

Question 4

Hyper IgM Syndrome

Answer 4

• Defect in CD40-CD40L, so can’t isotope switch.

- X-linked

Question 5

Primary B cell immunodeficiencies (list 4)

Answer 5

• Transient Hypogammaglobulinemia of infancy
• Common Variable Hypogammaglobulinemia
• Selective IgA Deficiency
• Hyper IgM Syndrome