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惰性白细胞综合征——粒细胞膜的疾病?

Lazy leucocyte syndrome--disorder of the granulocyte membrane?

作者信息

Pinkerton P H, Robinson J B, Senn J S

出版信息

J Clin Pathol. 1978 Apr;31(4):300-8. doi: 10.1136/jcp.31.4.300.

Abstract

An adult with long-standing neutropenia had the functional granulocyte abnormalities typical of the lazy leucocyte syndrome. Scanning electron microscopy of the patient's neutrophils showed alteration in the surface configuration of the cell with coarsening of the normal fine ruffles and the appearance of knob-like projections. Similar functional and anatomical changes were induced in normal neutrophils by treatment with vinblastine. The lazy leucocyte syndrome may be a consequence of altered membrane microfilamentous protein structure or function, and undue rigidity of the affected neutrophils may explain the clinicopathological features of the disease.

摘要

一名患有长期中性粒细胞减少症的成年人具有典型的惰性白细胞综合征的功能性粒细胞异常。对该患者的中性粒细胞进行扫描电子显微镜检查显示,细胞表面形态发生改变,正常的细微褶皱变粗,并出现瘤状突起。用长春碱处理正常中性粒细胞可诱导出类似的功能和结构变化。惰性白细胞综合征可能是膜微丝状蛋白结构或功能改变的结果,受影响的中性粒细胞过度僵硬可能解释了该疾病的临床病理特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2764/1145266/58d2b143b662/jclinpath00440-0006-a.jpg

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