Granulocyte Colony-Stimulating Factor???Responsive Chronic Neutropenia in Cartilage???Hair Hypoplasia:

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY(2004)

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摘要
Chronic neutropenia is common in children with cartilage hair hypoplasia (CHH). The authors describe a 6-year-old with severe CHH, moderate neutropenia associated with serum IgG antibodies directed against Fcgamma-RIIIb (NA 1/2), and frequent bacterial infections. In this patient, long-term administration of granulocyte colony-stimulating factor increased peripheral neutrophil counts and prevented recurrent hospitalizations for bacterial lower respiratory tract infections.
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关键词
cartilage-hair hypoplasia,immunodeficiency,autoimmune neutropenia
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