A Single Center Survey of Patients With Congenital Neutropenia: Report From Northwestern Iran



Sadeghishabestari M¹ ; Dousti S² ; Rezamand A² ; Harsini S^{3, 4} ; Rezaei N^{3, 4, 5} Show Affiliations
Source: Acta Medica Iranica Published:2018

Abstract

Neutropenia is characterized by a decrease in circulating neutrophil counts and consequent infections. The present study was performed to describe the clinical and laboratory findings of patients with congenital neutropenia in northwestern Iran. Medical 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 46.21±37.92 months, 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 13.16±12.48 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. © 2018 Tehran University of Medical Sciences. All rights reserved.