Congenital neutropenia syndromes

被引:23
作者
Boztug, Kaan [1 ]
Welte, Karl [1 ]
Zeidler, Cornelia [1 ]
Klein, Christoph [1 ]
机构
[1] Hannover Med Sch, Dept Pediat Hematol Oncol, D-30625 Hannover, Germany
关键词
D O I
10.1016/j.iac.2008.01.007
中图分类号
R392 [医学免疫学];
学科分类号
100102 ;
摘要
Congenital neutropenia syndromes comprise a heterogeneous group of inherited disorders. Hereditary conditions associated with low neutrophil counts are persistent and need to be differentiated from neutropenia secondary to autoimmune processes or other pathologic conditions, such as myelodysplasia or leukemia. Clinically, congenital neutropenia is characterized by recurrent bacterial infections. Recently, several novel genetic defects were described in patients with congenital neutropenia, shedding light on the pathophysiology of these rare diseases.
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页码:259 / +
页数:19
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