An infant with chronic severe neutropenia

Area of Science:

• Pediatrics
• Hematology
• Immunology

Background:

• Neutropenia in children presents diagnostic challenges due to diverse etiologies, ranging from acute to chronic conditions.
• Chronic benign neutropenia of infancy is a rare disorder affecting 1 in 100,000 infants, characterized by prolonged low neutrophil counts.

Observation:

• The case describes an infant with fever and cervical lymphadenitis, initially presenting a diagnostic challenge.
• The infant exhibited isolated severe neutropenia with persistent low neutrophil counts.

Findings:

• The infant experienced a prolonged course with minor infections but no serious underlying conditions.
• The clinical presentation and prolonged neutropenia, resolving spontaneously by age 3-4 years, confirmed the diagnosis of chronic benign neutropenia of infancy.

Implications:

• This case highlights the importance of considering chronic benign neutropenia of infancy in infants with persistent neutropenia and recurrent infections.
• Early diagnosis and monitoring can prevent unnecessary investigations and guide appropriate management for this generally benign condition.