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骨髓增生异常综合征中的粒细胞发育异常与功能障碍以及CD11/CD18缺陷

Granulocyte dysplasia and dysfunction, and CD11/CD18 defects in myelodysplastic syndromes.

作者信息

Mazzone A, Porta C, Fossati G, Gritti D, Mazzucchelli I, Ricevuti G

机构信息

Dipartimento di Medicina Interna e Terapia Medica, Università degli Studi di Pavia, I.R.C.C.S. Policlinico San Matteo, Italy.

出版信息

Leuk Lymphoma. 1996 Oct;23(3-4):267-75. doi: 10.3109/10428199609054829.

DOI:10.3109/10428199609054829
PMID:9031107
Abstract

In myelodysplastic syndromes (MDS), dysplastic changes in neutrophils are a common feature reflecting the total degree of bone marrow dysplasia. Furthermore, granulocyte function is abnormal, so that a high risk of life-threatening infections has been documented. In this review we shall focus on the defects of both granulocytes and their CD11b/CD18 glycoprotein complex, which regulate granulocyte adherence, locomotion, diapedesis and migration into inflammatory sites, in patients suffering from primary MDS. The defective surface membrane glycoprotein expression of myelodysplastic phagocytes is not only a useful diagnostic tool, but also a powerful prognostic one, since MDS patients with such defects present both an increased susceptibility to infections and a decreased survival. Moreover, the administration of colony-stimulating factors is known to be able to elicit long-lasting improvement in neutrophil count, CD11b/CD18 expression and function, marrow myeloid maturation, and possibly to decrease bacterial infections in MDS patients.

摘要

在骨髓增生异常综合征(MDS)中,中性粒细胞的发育异常改变是反映骨髓发育异常总体程度的常见特征。此外,粒细胞功能异常,因此有记录表明存在危及生命感染的高风险。在本综述中,我们将重点关注原发性MDS患者粒细胞及其调节粒细胞黏附、运动、穿壁和迁移至炎症部位的CD11b/CD18糖蛋白复合物的缺陷。骨髓发育异常的吞噬细胞表面膜糖蛋白表达缺陷不仅是一种有用的诊断工具,也是一种有力的预后指标,因为存在此类缺陷的MDS患者对感染的易感性增加且生存率降低。此外,已知给予集落刺激因子能够使中性粒细胞计数、CD11b/CD18表达和功能、骨髓髓系成熟得到持久改善,并可能减少MDS患者的细菌感染。

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