Author/Authors :
Aghamohammadi, Asghar Department of Pediatrics - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Cheraghi, Taher Department of Pediatrics - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Rezaei, Nima Tehran University of Medical Sciences, Tehran , Kanegane, Hirokazu Department of Pediatrics - Graduate School of Medicine - University of Toyama - Toyama, Japan , Abdollahzede, Sina Tehran University of Medical Sciences, Tehran , Talaei-Khoei, Mojtaba Tehran University of Medical Sciences, Tehran , Heidari, Golnaz Tehran University of Medical Sciences, Tehran , Zandieh, Fariborz Department of Pediatrics - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Moin, Mostafa Department of Pediatrics - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Miyawaki, Toshio Tehran University of Medical Sciences, Tehran
Abstract :
X-linked Agammaglobulinemia (XLA) is a hereditary immunodeficiency, characterized by
an early onset of recurrent bacterial infections, hypogammaglobulinemia and markedly reduced
B lymphocytes number.
In order to determine the association of neutropenia among Iranian patients with XLA,
hospital records of 30 patients with confirmed XLA in Children Medical Center Hospital,
were reviewed.
Eight out of 30 XLA patients (26.7%) developed neutropenia during the course of the disease.
In two patients, episodes of neutropenia were identified before or at the time of diagnosis
of XLA. Other six patients whom were not visited regularly and did not receive periodical immunoglobulin
replacement therapy experienced neutropenia after diagnosis of XLA.
Neutropenia in XLA is mainly associated with infection and is resolved with intravenous
immunoglobulin replacement and antibiotics therapy.
