title: XLA-associated neutropenia treatment: a case report and review of the literature.
abstract: X-linked agammaglobulinemia (XLA) is a primary B-cell deficiency syndrome with an incidence of 5 to 10 cases per million. The current treatment approach includes intravenous immunoglobulin and aggressive antibiotic regimens for infections. Besides recurrent infections, XLA patients may present with other manifestations, such as alopecia, enteropathy, amyloidosis, and neutropenia. Neutropenia, which has been shown in up to 25% of affected patients, might also contribute to the degree of severity of bacterial infections that have been reported in these cases. Here we present our experience with the granulocyte colony-stimulant factor, filgrastim (Neupogen), in the treatment of neutropenia in a 14-month-old child with XLA.
authors.affiliation: Department of Child Health, J. Otto Lottes Health Sciences Library, University of Missouri-Columbia, Columbia, MO 65212, USA.
authors.forename: Zachary David
authors.initials: ZD
authors.lastname: Jacobs
authors.forename: Jesus Ramon
authors.initials: JR
authors.lastname: Guajardo
authors.forename: Katherine Marie
authors.initials: KM
authors.lastname: Anderson
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000503:physiopathology; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000069585:Filgrastim; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000503:physiopathology; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D041121:Klebsiella oxytoca; D007711:Klebsiella pneumoniae; D009503:Neutropenia / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D011018:Pneumonia, Pneumococcal; D011994:Recombinant Proteins; D013296:Streptococcus pneumoniae
chemical_list: D000900:Anti-Bacterial Agents; D016756:Immunoglobulins, Intravenous; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000069585:Filgrastim