Acta Medica Iranica 2018. 56(6):372-378.

A Single Center Survey of Patients With Congenital Neutropenia: Report From Northwestern Iran
Mahnaz Sadeghi-Shabestari, Samaneh Dousti, Azim Rezamand, Sara Harsini, Nima Rezaei


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.


Neutropenia; Immunologic deficiency syndromes; Infection; Iran

Full Text:



Zafrani L, Azoulay E. How to treat severe infections in critically ill neutropenic patients? BMC infectious diseases. 2014;14:512.

Boxer L, Dale DC. Neutropenia: causes and consequences. Seminars in hematology. 2002;39(2):75-81.

Fioredda F, Calvillo M, Bonanomi S, Coliva T, Tucci F, Farruggia P, et al. Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica). Pediatric blood & cancer. 2011;57(1):10-7.

Farruggia P, Dufour C. Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians. Therapeutic advances in hematology. 2015;6(1):15-24.

Celkan T, Koc BS. Approach to the patient with neutropenia in childhood. Turk pediatri arsivi. 2015;50(3):136-44.

Watts R. Neutropenia. Wintrobe’s Clinical Hematology USA: Lippincott Williams and Wilkins. 1999:1862-88.

Nguyan D, Diamond L. Neutropenia/leukopenia pattern. Diagnostic hematology: A pattern approach Spain: Butterworth Heinemann. 2000:102-12.

Boxer LA, Newburger PE. A molecular classification of congenital neutropenia syndromes. Pediatric blood & cancer. 2007;49(5):609-14.

Aghamohammadi A, Moein M, Farhoudi A, Pourpak Z, Rezaei N, Abolmaali K, et al. Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults. Journal of clinical immunology. 2002;22(6):375-80.

Sokolic R. Neutropenia in primary immunodeficiency. Current opinion in hematology. 2013;20(1):55-65.

Cham B, Bonilla MA, Winkelstein J. Neutropenia associated with primary immunodeficiency syndromes. Seminars in hematology. 2002;39(2):107-12.

Shabestari MS, Maljaei SH, Baradaran R, Barzegar M, Hashemi F, Mesri A, et al. Distribution of primary immunodeficiency diseases in the Turk ethnic group, living in the northwestern Iran. Journal of clinical immunology. 2007;27(5):510-6.

Rezaei N, Farhoudi A, Ramyar A, Pourpak Z, Aghamohammadi A, Mohammadpour B, et al. Congenital neutropenia and primary immunodeficiency disorders: a survey of 26 Iranian patients. Journal of pediatric hematology/oncology. 2005;27(7):351-6.

Rezaei N, Farhoudi A, Pourpak Z, Aghamohammadi A, Moin M, Movahedi M, et al. Neutropenia in Iranian patients with primary immunodeficiency disorders. Haematologica. 2005;90(4):554-6.

Leale M. Recurrent furunculosis in an infant showing an unusual blood picture. Journal of the American Medical Association. 1910;54(23):1854-5.

Unsworth DJ, Thomas HM. Missed clues and delayed diagnosis of immunodeficiency. Lancet (London, England). 1997;349(9049):435.

Chapel HM. Consensus on diagnosis and management of primary antibody deficiencies. Consensus Panel for the Diagnosis and Management of Primary Antibody Deficiencies. BMJ (Clinical research ed). 1994;308(6928):581-5.


  • There are currently no refbacks.

Creative Commons Attribution-NonCommercial 3.0

This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.