ABSTRACT
Neutrophils play a vital role in protecting the body against invasion by bacte ria. Neutropenia predisposes a patient to bacterial infections. Severe congenital neutropenia is a general term that describes a condition of absolute neutro phil counts (ANCs) less than 500/pL present at birth or during infancy. Con genital neutropenia is caused by a variety of hematological, immunological, and metabolic diseases (Table 1). This chapter focuses on the severe forms of congenital neutropenia.