29. Immunodeficiency Diseases II

Question 1

What type of deficiency would a patient with a decreased number of CD3-positive cells have? List one example of this type of deficiency.

Answer 1

Deficiency in Cell-Mediated Immunity

Ex: DiGeorge Syndrome; Chronic Mucocutaneous

Question 2

What is the immunodeficiency in which patients have an underdeveloped thymus, underdeveloped parathyroid glands, congenital abnormalities of the heart, and abnormal facial features?

Answer 2

DiGeorge Syndrome

Question 3

What immunodeficiency disease results from a defect in lymphocyte stem cell precursors?

Answer 3

Severe Combined Immundeficiency Disease (SCID)

Question 4

What are the 3 characteristic clinical features of the Wiskott Aldrich syndrome?

Answer 4

1. Thrombocytopenia
2. Eczema
3. recurrent infections and high incidence of leukemias/lymphomas

Question 5

What is meant by ataxia? telangectasia?

Answer 5

Ataxia = incoordinationof voluntary muscles
Telangectasia = dialation of groups of capillaries

Question 6

What syndrome is also known as Hyperimmunoglobulinemia E?

Answer 6

Job’s Syndrome

Question 7

What immunodeficiency is characterized by decreased ability to produce hydrogen peroxide and superoxide anion?

Answer 7

Chronic Granulomatous Disease (CGD) a phagocytic cell defect

Question 8

Why are patients with Chronic Granulomatous Disease most susceptible to infections with catalase positive organisms?

Answer 8

Catalase positive organisms break down H2O2 produced by host or bacteria

Question 9

In what immunodeficiency would you expect to see decreased NBT dye and DHR reduction test results?

Answer 9

Chronic Granulomatous Disease (CGD)

Question 10

What immunodeficiency is characterized by defective, giant lysosomal granules?

Answer 10

Chediak Higashi Syndrome