National Consensus Guideline on Diagnosis and Management of Congenital Neutropenias

Saba Fekrvand; Reza Yazdani; Samaneh Abdolahzade; Parisa Ashournia; Aida Askarisarvestani; Sima Bahrami; Maryam Behfar; Zahra Daneshmandi; Taher Cheraghi; Sepideh Darougar; Sarehsadat Ebrahimi; Shabnam Eskandarzadeh; Narges Eslami; Golnaz Eslamian; Shahrzad Fallah; Ali Haghbin; Mehrnaz Mesdaghi; Majid Marjani; Mohammadreza Modaresi; Mahshid  Movahedi; Mohammamd Nabavi; Anahita Razaghian; Mahnaz  Sadeghi-Shabestari; Sahar Seraj; Seyedehshabnam Seyedsalehi; Alireza Shafiei; Reza Shiari; Sima Shokri; Saman Tavakoli; Ahmad Vosughimotlagh; Vahid Ziaee; Nima Rezaei

doi:10.18502/igj.v8i3.18171

Saba Fekrvand Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Science, Tehran, Iran
Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Science, Tehran, Iran
Samaneh Abdolahzade Department of Pediatric disease, School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran
Parisa Ashournia Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Aida Askarisarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; Assistant Professor, Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Zahra Daneshmandi Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran
Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran
Sarehsadat Ebrahimi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Shabnam Eskandarzadeh Department of pediatrics, Pediatric Health Center, Tabriz University of Medical Sciences, Tabriz, Iran
Narges Eslami Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Golnaz Eslamian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Shahrzad Fallah Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran
Ali Haghbin Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran
Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center Hospital, Tehran, Iran
Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Mohammamd Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
Anahita Razaghian Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Mahnaz Sadeghi-Shabestari Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran
Sahar Seraj Department of Immunology and Allergy, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Seyedehshabnam Seyedsalehi Abuzar Children’s Medical Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
Alireza Shafiei Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
Reza Shiari Division of Pediatric Rheumatology Department of Pediatrics Mofid Children's Hospital Shahid Beheshti University of Medical Sciences, Tehran, Iran
Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
Saman Tavakoli Department of Pediatric, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Ahmad Vosughimotlagh Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Vahid Ziaee Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Science, Tehran, Iran

DOI: https://doi.org/10.18502/igj.v8i3.18171

Keywords: National consensus; Inborn errors of immunity; Severe congenital neutropenia; Management; G-CSF; HSCT.

Abstract

At present, a national consensus or guideline for diagnosing and managing patients suspected of having se- vere congenital neutropenia (SCN) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunolo- gists reviewed the current consensus, presented their comments at a meeting titled “First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts” and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, administration of granulocyte colony-stimulating factor (G-CSF) and hematopoietic stem cell transplantation (HSCT) for patients with SCN.