Granulocyte colony-stimulating factor-responsive chronic neutropenia in cartilage-hair hypoplasia

被引：6

作者：

Ammann, RA

Duppenthaler, A

Bux, J

Aebi, C

机构：

[1] Univ Bern, Dept Pediat, Bern, Switzerland

[2] Univ Bern, Inst Infect Dis, Bern, Switzerland

[3] Swiss Red Cross, Transfus Serv, Bern, Switzerland

来源：

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY | 2004年 / 26卷 / 06期

关键词：

cartilage-hair hypoplasia; immunodeficiency; autoimmune neutropenia;

D O I：

10.1097/00043426-200406000-00009

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

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.

引用

收藏

页码：379 / 381

页数：3

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