Abstract
“Neutropenia” generally indicates a blood pathology in which the concentration of neutrophils in the peripheral blood is lower than 1500/mm3. Kostmann syndrome is a rare congenital severe neutropenia and is characterized by recurrent bacterial infections, fever, skin infection, gingivitis and early life-threatening infections. We report a 6–year-old girl with Kostmann syndrome who suffered from neck infection and swelling in the epiglottis without pain. She needed a daily dose of antibiotics to prevent infection. She visited to our hospital complaining of a cough. Four days after the visit, she started to experience fever and hoarseness, and one week after the visit, she returned with a right submandibular mass. Computed tomography revealed a low attenuation mass in the right parapharyngeal space without ring enhancement. A fexible laryngoscope study revealed swelling in the epiglottis and right arytenoid cartilag. Based on these findings the patient was diagnosed with a deep neck infection, and intravenous administration of antibiotics (meropenem), steroid and G–CSF (granulocyte colony-stimulating factor) was started immediately, following which a gradual improvement insymptoms was observed. However, swelling in the epiglottis remained unchanged.
