Primary antibody deficiency in a tertiary referral hospital: A 30-year experiment

Mohammadinejad, P. and Pourhamdi, S. and Abolhassani, H. and Mirminachi, B. and Havaei, A. and Masoom, S.N. and Sadeghi, B. and Ghajar, A. and Afarideh, M. and Parvaneh, N. and Mirsaeed-Ghazi, B. and Movahedi, M. and Gharagozlou, M. and Chavoushzadeh, Z. and Mahdaviani, A. and Zandieh, F. and Sherkat, R. and Sadeghi-Shabestari, M. and Faridhosseini, R. and Jabbari-Azad, F. and Ahanchian, H. and Zandkarimi, M. and Cherghi, T. and Fayezi, A. and Mohammadzadeh, I. and Amin, R. and Aleyasin, S. and Moghtaderi, M. and Ghaffari, J. and Bemanian, M. and Shafiei, A. and Kalantari, N. and Ahmadiafshar, A. and Khazaei, H.A. and Mohammadi, J. and Nabavi, M. and Rezaei, N. and Aghamohammadi, A. (2015) Primary antibody deficiency in a tertiary referral hospital: A 30-year experiment. Journal of Investigational Allergology and Clinical Immunology, 25 (6). pp. 416-425.

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Abstract

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. © 2015 Esmon Publicidad.

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