Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 56 6 2018 06 23 372 378 EN Mahnaz Sadeghi-Shabestari Immunology and Allergy Research Center of Tabriz, Children's Hospital, Tuberculosis and Lung Research Center of Tabriz, Tabriz University of Medical Sciences, Tabriz, Iran. Samaneh Dousti Department of Pediatrics, Children’s Hospital, Tabriz University of Medical Sciences, Tabriz, Iran. Azim Rezamand Department of Pediatrics, Children’s Hospital, Tabriz University of Medical Sciences, Tabriz, Iran. Sara Harsini Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran. Nima Rezaei Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran. AND Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. 2017 05 28 2018 01 17 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. https://acta.tums.ac.ir/index.php/acta/article/view/6486 https://acta.tums.ac.ir/index.php/acta/article/download/6486/5106