Page 508 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHAPtER 34 Primary Antibody Deficiencies 487.e1

M u L t IPLE-CH o ICE Q u ES t I o NS

1. A 50-year-old man presents with a history of two pneumonias smoked, and testing for cystic fibrosis is negative. She com-
in the last 2 years. He has suffered occasionally with sinusitis plains of dyspnea on climbing more than one flight of stairs
and bronchitis since childhood, but these infections have and suffers with fatigue. She suffered pneumonia once as a
readily responded to antibiotic therapy, and he denies dyspnea child. What would be the best approach?
on exertion. A grandson is receiving therapy for an unknown A. The patient has selective IgA deficiency. She should be
immune deficiency. Serum immunoglobulin (Ig) levels are treated with antibiotics, as needed, for infections.
drawn. IgM is <20 g/dL. IgG is 100 mg/dL. IgA is <7 mg/dL. B. The patient may have an IgG subclass deficiency in addition
Flow cytometry reveals rare B cells. He has no response to to IgA deficiency. She should be treated with prophylactic
pneumococcal polysaccharide vaccination. What is the most antibiotics, since gammaglobulin replacement is contra-
likely diagnosis? indicated for patients with IgA deficiency.
A. Hyper-IgM syndrome C. The patient may have an IgG subclass deficiency in addition
B. Common variable immune deficiency to IgA deficiency. She should be treated with gammaglobulin
C. X-linked agammaglobulinemia replacement therapy.
D. Autosomal agammaglobulinemia
3. A 2-month-old baby girl presents with her second episode
2. A 35-year-old woman presents with a 10-year history of of bacterial pneumonia. Serum immunoglobulin (Ig) levels
recurrent sinopulmonary infections. She has suffered with are drawn. IgM is 100 g/dL. IgG is 100 mg/dL. IgA is <7 mg/
allergic rhinitis since childhood and was diagnosed with celiac dL. Flow cytometry reveals normal B-cell numbers. What is
disease at age 15 years. Serum immunoglobulin (Ig) levels the most likely diagnosis?
are drawn. IgM is 100 g/dL. IgG is 650 mg/dL. IgA is <7 mg/ A. Selective IgA deficiency
dL. Flow cytometry reveals normal B-cell numbers. After B. Transient hypogammaglobulinemia of infancy
vaccination with Pneumovax23, she responds to only five of C. Common variable immune deficiency
23 pneumococcal polysaccharide antigens. She has never D. Maternal common variable immune deficiency