HY USMLE Q #666 – Immuno

A 3-year-old boy is brought to the physician for a 1-day history of fever and mouth sores. He has a 12-month history of similar episodes lasting 3 to 5 days that occur every 3 weeks. Temperature is 38.5 C. Physical examination shows various aphthous ulcers within the oral cavity. Laboratory studies show:

Hemoglobin                                                                       14 g/dL
Leukocytes                                                                         4000/μL
Neutrophils                                                                        5%
Lymphocytes                                                                     85%
Monocytes                                                                         10%
Platelets                                                                               300,000/μL

Which of the following is most likely responsible for this patient’s presentation?

Chédiak-Higashi syndrome
ELANE gene mutation
Fanconi anemia
Severe congenital neutropenia
Viral-induced neutropenia