Author/Authors :
Atarod, Lida Department of Allergy and Clinical Immunology - Immunology - Asthma and Allergy Research Institute - Tehran University of Medical Sciences, Tehran , Aghamohammadi, Asghar Department of Allergy and Clinical Immunology - Immunology - Asthma and Allergy Research Institute - Tehran University of Medical Sciences, Tehran , Moin, Mostafa Department of Allergy and Clinical Immunology - Immunology - Asthma and Allergy Research Institute - Tehran University of Medical Sciences, Tehran , Kanegane, Hiro Department of Pediatrics - Faculty of Medicine - Toyama Medical and Pharmaceutical University, Toyama, Japan , Rezaei, Nima Department of Allergy and Clinical Immunology - Immunology - Asthma and Allergy Research Institute - Tehran University of Medical Sciences, Tehran , Rezaei Kalantari, Kiara Department of Allergy and Clinical Immunology - Immunology - Asthma and Allergy Research Institute - Tehran University of Medical Sciences, Tehran , Amirzagar, Ali Akbar Department of Immunology of School of Medicine - Tehran University of Medical Sciences, Tehran , Futatani, Takishi Department of Pediatrics - Faculty of Medicine - Toyama Medical and Pharmaceutical University, Toyama, Japan , Miyawaki, Toshio Department of Pediatrics - Faculty of Medicine - Toyama Medical and Pharmaceutical University, Toyama, Japan
Abstract :
Hyper-IgM syndromes are characterized by profound reduction of serum IgG, IgA, and
IgE levels with normal or increased concentrations of serum IgM. CD40 ligand deficiency is
X-linked form of the disease, which results in a lack of immunoglobulin class switching from
IgM to IgG in B cells. In addition to the recurrent infections, a number of patients suffer
from neutropenia. There are some evidences indicating the effect of G-CSF in combination
with intravenous immunoglobulin (IVIG) in improvement of neutrophil counts, which has
become the most common procedure to control neutropenia.
In this report we present a 6 year-old patient of CD40 ligand deficiency, who suffered
from chronic, severe neutropenia. Administration of IVIG was started for him when the
diagnosis was made at the age of 1.5 years and he was on the regular IVIG therapy after that
time untill now for a period of 4.5 years. IVIG and prophylactic antibiotic therapy, despite
cessation of granulocyte colony-stimulating factor, injection after one month, corrected the
severe neutropenic state of this patient.
It seems that regular administration of sufficient doses of IVIG can be useful in the
management of neutropenia in CD40 ligand deficiency, which results in better quality of life
with decreasing occurrence of infection.
Keywords :
Antibody Deficiency , CD40 Ligand , Hyper IgM Syndrome , Intravenous Immunoglobulin , Neutropenia
