Immunology Review Corner

Immunology Review Corner: Chapter 59, Part II of the 6th edition of Middleton’s Allergy Principles and Practice, edited by N. Franklin Adkinson, et al.

Prepared by Dr. Thao N. Tran, Puget Sound Allergy, Asthma and Immunology, and Dr. Karla L. Davis, Walter Reed Medical Center.

Chapter 59:  Primary Immunodeficiency Diseases – Part II

1. Which of the following laboratory findings is usually normal or increased in patients with Ataxia-Telangiectasia?
a. IgA concentration
b. IgE concentration
c. CD8+ T cell concentration
d. Proliferative response to mitogens

2. Which of the following conditions is notable for radiologic findings of scalloping and sclerotic or cystic changes in the metaphyses?
a. Ataxia-Telangiectasia
b. Cartilage-Hair Hypoplasia
c. DiGeorge Syndrome
d. Chediak-Higashi Syndrome

3. A young girl with recurrent pneumonia has elevated IgM and low to undetectable levels of IgG, IgA, and IgE. What is most likely the defective gene?
a. Activation-Induced Cytidine Deaminase
b. Purine Nucleoside Phosphorylase
c. CD154 (CD40Ligand)
d. CD11/CD18

4. Which of the following statements about DiGeorge Syndrome is the most accurate?
a. There appears to be an excess of 22q11.2 deletions of paternal origin
b. Proportions of CD4+ and CD8+ cells are normal.
c. Most T lymphocytes present are intrinsically abnormal.
d. CD3+T cell numbers are increased

5. The patient presents with delayed separation of the umbilical cord and recurrent severe bacterial infections. You plan to order cytofluorography with monoclonal antibody to:
a. Ig-alpha
b. CD80/86
c. Sialyl-LewisX
d. CD18

6. GDP-fucose transporter deficiency is a cause of which of the following immunodeficiencies?
a. Leukocyte Adhesion Deficiency Type 1
b. Leukocyte Adhesion Deficiency Type 2
c. Severe Combined Immunodeficiency
d. Wiskott-Aldrich Syndrome

7. Which of the following conditions is characterized by oculocutaneous albinism and susceptibility to recurrent respiratory tract infections?
a. Ataxia-Telangiectasia
b. Cartilage-Hair Hypoplasia
c. Chediak-Higashi Syndrome
d. Hyperimmunoglobulinemia E Syndrome

8. Serum concentrations of other immunogloblulins are usually normal in patients with Selective Immunoglobulin A Deficiency. However, which of the following IgG subclass deficiencies may be present?
a. IgG1
b. IgG2
c. IgG3
d. IgG4

9. A patient presents with course facial features, recurrent severe staphylococcal abscesses of the skin and lung, and pneumatoceles on chest x-ray. Which of the following lab findings will most likely be significantly elevated?
a. IgE
b. IgM
c. Serum neutrophils
d. Gamma-delta T cells

10. What is the most common inherited complement deficiency?
a. C2
b. C3
c. C5
d. Properdin

Answers
1. c, page 1028
2. b, page 1028
3. a, page 1029
4. b, page 1029
5. d, page 1030
6. b, page 1030
7. c, page 1031
8. b, page 1032
9. a, page 1035
10. a, pages 1035-36