title: Severe neutropenia in children: a single institutional experience.
abstract: Severe neutropenia (SN) is a rare disorder in childhood. This study aimed to document the approach to diagnosis and treatment of children with SN in a single university-based children's hospital, determine the types of SN seen in a 4-year period, and determine outcomes of the subtypes of SN. Forty-five children with SN were identified between 2000 and 2004. Two patients had autoimmune, 3 congenital, 3 familial, 6 cyclic, and 31 idiopathic SN. The median age of the patients with idiopathic SN was 15 months (3 mo to 17 y). Thirteen patients with idiopathic SN received filgrastim and 18 were observed. The history of severe infection and hospitalization at presentation was significantly more common among the patients who received filgrastim than those observed, but was not different between the 2 groups during the follow-up period. SN resolved in 16 patients and persisted in 14 patients. One patient with idiopathic SN did not respond to filgrastim and died of sepsis while she was still neutropenic. In summary, the majority of patients with SN had idiopathic SN, the infection risk was variable, treatment was based on clinical judgment rather than absolute neutrophil count, and approximately half of the patients had complete recovery.
authors.affiliation: Department of Pediatric Hematology, Ege University Faculty of Medicine, Izmir, Turkey. dyilmazk@yahoo.com
authors.forename: Deniz
authors.initials: D
authors.lastname: Yilmaz
authors.forename: Arthur Kim
authors.initials: AK
authors.lastname: Ritchey
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D000069585:Filgrastim; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D011994:Recombinant Proteins; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000069585:Filgrastim