Neutropenia is characterized by a decrease in circulating neutrophil counts and consequent infections. The present study was performed so as to describe the clinical and laboratory findings of patients with congenital neutropenia in northwestern Iran. The patients' records of 31 patients with congenital neutropenia out of 280 neutropenic patients who had been referred to Tabriz Children's Hospital during a 3-year period (2011-2014), were reviewed. Thirty-one cases (17 female and 14 male), with a mean age of 5.3 ± 5.7 years, were diagnosed to suffer from congenital neutropenia. The disorders associated with congenital neutropenia were combined immunodeficiency (8 cases), severe congenital neutropenia (6 cases), common variable immunodeficiency (4 cases), severe combined immunodeficiency (2 cases) and metabolic syndrome (1 case). The median age of the onset of disease was 26.2 ± 60.8 months. The most common clinical manifestations during the course of illness were otitis media (13 cases), pneumonia (12 cases), recurrent aphthous stomatitis, lymphadenopathy and gingivitis (11 cases). Four neutropenic patients died because of recurrent infections. Neutropenia may occur in the context of the primary immunodeficiency disorders. Unusual, persistent or severe infections always pose a speculation to search for an underlying immunodeficiency syndrome and neutropenia, so as to avoid further life-threatening complications as a result of any delay in diagnosis
