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Childhood onset cyclic neutropenia: G‐CSF therapy restores neutrophil count but does not influence superoxide anion and cytokine release by neutrophils
Author(s) -
Marcolongo Renzo,
Zambello Renato,
Trentin Livio,
Cassatella Marco,
Gasparotto Giuseppe,
Agostini Carlo
Publication year - 1995
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1995.tb03301.x
Subject(s) - superoxide , neutropenia , cyclic neutropenia , medicine , immunology , cytokine , neutrophil extracellular traps , granulocyte colony stimulating factor , congenital neutropenia , granulocyte , neutrophile , absolute neutrophil count , inflammation , chemistry , chemotherapy , biochemistry , enzyme
Summary. In this paper we describe the case of a 16‐year‐old boy with childhood onset cyclic neutropenia (CN) with a 21d cycle who was successfully treated with recombinant granulocyte‐colony stimulating factor (G‐CSF). Cyclic therapy with G‐CSF (5μg/kg/d s.c. for 1 week every 21d) 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‐1/3, IL‐8, TNFα) and superoxide anion during G‐CSF therapy.