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一对患有低丙种球蛋白血症的父女患有一种罕见形式的慢性中性粒细胞减少症。

An unusual form of chronic neutropenia in a father and daughter with hypogammaglobulinaemia.

作者信息

Mentzer W C, Johnston R B, Baehner R L, Nathan D G

出版信息

Br J Haematol. 1977 Jul;36(3):313-22. doi: 10.1111/j.1365-2141.1977.tb00654.x.

DOI:10.1111/j.1365-2141.1977.tb00654.x
PMID:889707
Abstract

Chronic neutropenia, with an abundance of mature neutrophils in the bone marrow, was discovered in a father and daughter who also had common variable hypogammaglobulinaemia. Under the stress of infection or following the administration of typhoid vaccine, release of neutrophils from the marrow was enhanced sufficiently to abolish neutropenia temporarily. Although their morphology was abnormal, the function of neutrophils in vitro was normal. Thus, the increased susceptibility to infection that characterized these patients appeared to be due primarily to their defect in humoral immunity rather than their neutropenia.

摘要

在一位父亲及其女儿身上发现了慢性中性粒细胞减少症,其骨髓中有大量成熟的中性粒细胞,他们还患有常见变异型低丙种球蛋白血症。在感染应激状态下或接种伤寒疫苗后,骨髓中中性粒细胞的释放得到充分增强,足以暂时消除中性粒细胞减少症。尽管这些中性粒细胞的形态异常,但它们在体外的功能正常。因此,这些患者易感染的特征似乎主要是由于其体液免疫缺陷,而非中性粒细胞减少症。

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