Neutropenia associated with X-linked Agammaglobulinemia in an Iranian referral center.
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.
Aghamohammadi
Asghar
A
Department of Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@sina.tums.ac.ir
Cheraghi
Taher
T
Rezaei
Nima
N
Kanegane
Hirokazu
H
Abdollahzede
Sina
S
Talaei-Khoei
Mojtaba
M
Heidari
Golnaz
G
Zandieh
Fariborz
F
Moin
Mostafa
M
Miyawaki
Toshio
T
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
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D011505:Protein-Tyrosine Kinases; D000077329:Agammaglobulinaemia Tyrosine Kinase