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Treatment

The only simple treatment here is passive administration of pooled immunoglobulin. In the future, as biotechnology develops and clinical applicability of our current knowledge becomes the norm, we may be able to offer gene transfer technology (giving this child back the defective CD40 ligand gene). For now, as he prepares to travel to an area of HepB endemicity, he should certainly receive passive HepB immune globulin for protection!

Test Yourself

Multiple Choice Questions

For questions 1-3 make the best match with one of the following. Use each choice once, more than once, or not at all.

d. ITIM

1. Mutations in this protein leads to X-linked aggamagobulinemia

2. Activation motif

3. Present on FcyRIIB

4. All the following occur in germinal centers EXCEPT

a. differentiation to plasma cells b. affinity maturation (somatic mutation)

c. isotype switching d. memory cell formation e. somatic recombination

5. Mrs. L, a 37-year-old housewife with no history of childhood illness, has had recurrent chest and pyogenic infections over the last 18 months. She appears unwell and is found to be anemic. Tests indicate low levels of circulating immunoglobulins and she failed to produce specific antibodies to a booster dose of tetanus toxoid given one month previously. What is the most likely diagnosis?

a. x-linked hypogammaglobulinemia b. common variable hypogammaglobulinemia c. transient hypogammaglobulinemia of infancy d. selective IgA deficiency e. could be any one of the above

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