title: 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.affiliation: Department of Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@sina.tums.ac.ir
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Taher
authors.initials: T
authors.lastname: Cheraghi
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kanegane
authors.forename: Sina
authors.initials: S
authors.lastname: Abdollahzede
authors.forename: Mojtaba
authors.initials: M
authors.lastname: Talaei-Khoei
authors.forename: Golnaz
authors.initials: G
authors.lastname: Heidari
authors.forename: Fariborz
authors.initials: F
authors.lastname: Zandieh
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
authors.forename: Toshio
authors.initials: T
authors.lastname: Miyawaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000077329:Agammaglobulinaemia Tyrosine Kinase; D000361:Agammaglobulinemia / Q000097:blood / Q000150:complications* / Q000473:pathology; D017668:Age of Onset; D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003141:Communicable Diseases / Q000150:complications / Q000473:pathology / Q000628:therapy; D040181:Genetic Diseases, X-Linked / Q000097:blood / Q000150:complications* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D007223:Infant; D007492:Iran; D007958:Leukocyte Count; D018655:Lymphocyte Count; D009503:Neutropenia / Q000150:complications* / Q000453:epidemiology / Q000209:etiology; D009504:Neutrophils / Q000473:pathology; D011505:Protein-Tyrosine Kinases / Q000235:genetics / Q000378:metabolism; D055815:Young Adult
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D011505:Protein-Tyrosine Kinases; D000077329:Agammaglobulinaemia Tyrosine Kinase