Page 354 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHaPtEr 22 Phagocyte Deficiencies 333.e1

MUL t IPLE-CH o ICE QUES t I o NS

1. A 12-year-old boy with X-linked chronic granulomatous A. Tissue neutropenia leading to severe bacterial infection
disease (CGD) presents to your office with progressive fever, B. Excessive interferon (IFN)-γ production
dyspnea, and hypoxia a week after a Halloween hayride at a C. Poor root maintenance by defective macrophages
local farm. His chest X-ray shows extensive bilateral military D. Excessive interleukin (IL)-17 production
infiltrates. The MOST likely diagnosis and management: E. Severe tooth decay caused by impaired neutrophil trafficking
A. Burkholderia cepacia complex pneumonia; initiate 3. GATA2 deficiency is a complex disease with manifestations
antibacterials involving hematopoietic and lymphatic development. It is
B. Nocardia spp. pneumonia; initiate antibacterials often identified in adolescence or adulthood with cytopenias
C. Serratia marcescens pneumonia; initiate antibacterials and/or infections. Signs and symptoms that should lead to
D. Mulch pneumonitis; initiate antifungals and corticosteroids early consideration of GATA2 deficiency include:
E. Staphylococcus aureus bacteremia; initiate antibacterials A. Human papilloma virus (HPV) infection, mycobacterial
and obtain echocardiogram
infection, monocytopenia
2. Leukocyte adhesion defect-1 (LAD-1) leads to low numbers B. Respiratory syncytial virus (RSV) infection, staphylococcal
of neutrophils in the tissues (tissue neutropenia) and recurrent pneumonia, monocytosis
severe infections. Progressive severe periodontitis is charac- C. Influenza infection, Aspergillus infection, thrombocytopenia
teristic, with most patients losing all their teeth by late D. Adenovirus infection, Bordetella infection, anemia
adolescence. The immunological mechanism behind the E. Cytomegalovirus (CMV) infection, streptococcal infection,
periodontal disease is: lymphocytosis