骨髓增生异常综合征中性粒细胞上1型补体受体（CR1）缺乏

Complement receptor type 1 (CR1) deficiency on neutrophils in myelodysplastic syndrome.

作者信息

Ohsaka A, Saionji K, Watanabe N, Yokomichi H, Sugahara Y, Nagayama R, Igari J

机构信息

Department of Internal Medicine, Hitachi General Hospital, Ibaraki, Japan.

出版信息

Br J Haematol. 1994 Oct;88(2):409-12. doi: 10.1111/j.1365-2141.1994.tb05042.x.

DOI:10.1111/j.1365-2141.1994.tb05042.x

PMID:7803293

Abstract

We present a patient with myelodysplastic syndromes (MDS) whose neutrophils exhibited defective expression of complement receptor type 1 (CR1). A 73-year-old man was admitted with an evolution of MDS from RA into RAEBT according to the FAB classification of MDS. The neutrophil alkaline phosphatase (NAP) score was zero. The surface expression of membrane effector molecules on neutrophils was determined by indirect immunofluorescence using flow cytometry and monoclonal antibodies. The expression of CR1 on neutrophils as identified by staining with CD35 was defective in the patient, and the expression of other complement receptors (CR3 and CR4), Fc receptors and adhesion molecules was normal. CR1 deficiency and defective NAP score on neutrophils in the patient might account for impairment of common storage pool, presumably novel intracellular secretory vesicles.

摘要

我们报告了一名骨髓增生异常综合征（MDS）患者，其嗜中性粒细胞表现出补体受体1型（CR1）表达缺陷。一名73岁男性因根据MDS的FAB分类，从RA型MDS进展为RAEBT型MDS而入院。中性粒细胞碱性磷酸酶（NAP）评分为零。使用流式细胞术和单克隆抗体通过间接免疫荧光法测定嗜中性粒细胞上膜效应分子的表面表达。通过用CD35染色鉴定的患者嗜中性粒细胞上CR1的表达存在缺陷，而其他补体受体（CR3和CR4）、Fc受体和粘附分子的表达正常。患者嗜中性粒细胞中的CR1缺陷和NAP评分缺陷可能解释了共同储存池（可能是新型细胞内分泌小泡）的损伤。