Neutropenia associated with primary immunodeficiency syndromes

被引:55
作者
Cham, B
Bonilla, MA
Winkelstein, J
机构
[1] Univ Manitoba, Cancercare Manitoba, Dept Pediat, Winnipeg, MB R3V 0V9, Canada
[2] St Barnabas Hosp, Livingston, NJ USA
[3] Johns Hopkins Univ, Sch Med, Dept Pediat, Baltimore, MD 21205 USA
[4] Johns Hopkins Univ, Sch Med, Dept Med, Baltimore, MD 21205 USA
[5] Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA
关键词
D O I
10.1053/shem.2002.31916
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The primary immunodeficiency diseases are a heterogeneous group of more than 75 disorders characterized by intrinsic defects in the functions of the immune system. Many are associated with abnormalities of hematopoiesis as well. This article will review those primary immunodeficiency syndromes in which neutropenia is a prominent finding, including X-linked agammaglobulinemia (XLA), hyper IgM syndrome, common variable immunodeficiency (CVID), IgA deficiency, cartilage-hair hypoplasia (CHH), and reticular dysgenesis, with regards to pathophysiologic findings and treatment. Copyright 2002, Elsevier Science (USA). All rights reserved.
引用
收藏
页码:107 / 112
页数:6
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