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Original Article


Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment


Mohammadinejad P1, Pourhamdi S1, Abolhassani H1,2, Mirminachi B1, Havaei A1, Masoom SN1, Sadeghi B1, Ghajar A1, Afarideh M1, Parvaneh N1, Mirsaeed-Ghazi B3, Movahedi M4, Gharagozlou M4, Chavoushzadeh Z5, Mahdaviani A6, Zandieh F3, Sherkat R7, Sadeghi-Shabestari M8, Faridhosseini R9, Jabbari-Azad F9, Ahanchian H9, Zandkarimi M9, Cherghi T10, Fayezi A11, Mohammadzadeh I12, Amin R13, Aleyasin S13, Moghtaderi M13, Ghaffari J14, Bemanian M15, Shafiei A3,15, Kalantari N16, Ahmadiafshar A17, Khazaei HA18, Mohammadi J19, Nabavi M20, Rezaei N1,21, Aghamohammadi A1

1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
2Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, Stockholm, Sweden
3Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
4Department of Allergy and Clinical Immunology, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
5Pediatric Infectious Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
6Pediatric Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Disease, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
7Acquired Immunodeficiency Research Center, Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
8Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran
9Allergy Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
10Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran
11Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran
12Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran
13Department of Pediatric Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
14Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran
15Department of Immunology and Allergy, Yazd University of Medical Sciences, Yazd, Iran
16Department of Immunology and Allergy, Golestan University of Medical Sciences, Gorgan, Iran
17Department of Immunology and Allergy, Ghazvin University of Medical Sciences, Ghazvin, Iran
18Department of Immunology and Hematology, Zahedan Medical Sciences University, Zahedan, Iran
19Department of Life Science, Faculty of New Science and Technology, University of Tehran, Tehran, Iran
20Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
21Molecular Immunology Research Center and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

J Investig Allergol Clin Immunol 2015; Vol. 25(6): 416-425



Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease.

Objectives: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD.

Materials and Methods: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children’s Medical Center, Tehran, Iran.

Results: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients.

Conclusions: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.

Key words: Common variable immunodeficiency. Immunoglobulin A deficiency. Infection. Hyper-IgM syndrome. X-linked agammaglobulinemia.