Childhood onset cyclic neutropenia: G-CSF therapy restores neutrophil count but does not influence superoxide anion and cytokine release by neutrophils.
In this paper we describe the case of a 16-year-old boy with childhood onset cyclic neutropenia (CN) with a 21 d cycle who was successfully treated with recombinant granulocyte-colony stimulating factor (G-CSF). Cyclic therapy with G-CSF (5 micrograms/kg/d s.c. for 1 week every 21 d) maintained peripheral neutrophil count above the normal range, reduced the incidence of severe infections and significantly improved the patient's performance status throughout an 18-month follow-up. Phenotypic analysis of circulating lymphocytes demonstrated that G-CSF treatment does not modify the phenotypic profile of circulating B, T and NK cell populations. Circulating neutrophils released normal amounts of cytokines (including IL-1 beta, IL-8, TNF alpha) and superoxide anion during G-CSF therapy.
Marcolongo
R
R
Padua University School of Medicine, Department of Clinical Medicine, Italy.
Zambello
R
R
Trentin
L
L
Cassatella
M
M
Gasparotto
G
G
Agostini
C
C
D000293:Adolescent; D016207:Cytokines / Q000378:metabolism; D005500:Follow-Up Studies; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D016130:Immunophenotyping; D007958:Leukocyte Count; D016131:Lymphocyte Subsets; D008297:Male; D009503:Neutropenia / Q000378:metabolism / Q000628:therapy*; D009504:Neutrophils / Q000378:metabolism; D010507:Periodicity; D013481:Superoxides / Q000378:metabolism
D016207:Cytokines; D013481:Superoxides; D016179:Granulocyte Colony-Stimulating Factor