Neutropenia associated with X-linked Agammaglobulinemia in an Iranian referral center.

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.
Authors	Asghar Aghamohammadi, Taher Cheraghi, Nima Rezaei, Hirokazu Kanegane, Sina Abdollahzede, Mojtaba Talaei-Khoei, Golnaz Heidari, Fariborz Zandieh, Mostafa Moin, Toshio Miyawaki
Journal	Iranian journal of allergy, asthma, and immunology (Iran J Allergy Asthma Immunol) Vol. 8 Issue 1 Pg. 43-7 (Mar 2009) ISSN: 1735-1502 [Print] Iran
PMID	19279358 (Publication Type: Journal Article)
Chemical References	Immunoglobulin A Immunoglobulin G Immunoglobulin M Protein-Tyrosine Kinases Agammaglobulinaemia Tyrosine Kinase
Topics	Adolescent Adult Agammaglobulinaemia Tyrosine Kinase Agammaglobulinemia (blood, complications, pathology) Age of Onset B-Lymphocytes (pathology) Child Child, Preschool Communicable Diseases (complications, pathology, therapy) Genetic Diseases, X-Linked (blood, complications, pathology) Humans Immunoglobulin A (blood) Immunoglobulin G (blood) Immunoglobulin M (blood) Infant Iran Leukocyte Count Lymphocyte Count Neutropenia (complications, epidemiology, etiology) Neutrophils (pathology) Protein-Tyrosine Kinases (genetics, metabolism) Young Adult

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