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[副蛋白血症性成血病中的血小板糖胺聚糖]

[Platelet glycosaminoglycans in paraproteinemic hemoblastoses].

作者信息

Kharchenko M F, Bitiukova E S, Bessmel'tsev S S

出版信息

Vopr Med Khim. 1995 Nov-Dec;41(6):55-8.

PMID:8619307
Abstract

The content and composition of platelet glycosaminoglycans (GAG) were studied in 24 patients with paraproteinemic hemoblastoses (22 patients with myelomic disease and 2 with Waldenstrom's disease). According to the GAG levels, the patients were divided into 2 groups: 1) 6 patients with its higher levels (181 +/- 8 micrograms of uronic acids per 100 mg of acetone-dried cells) and 2) 18 patients with its close-to-normal levels (129 +/- 4 micrograms per 100 mg of the cells). Marked manifestations of hemorrhagic diathesis were found in 5 patients in Group 1 and in 1 in Group 2. Chondroitin sulfate was demonstrated to prevalent in the platelets of patients with paraproteinemic hemoblastoses, as in those of healthy persons. There was GAG electrophoretic profile depletion due to the reduced number of minor components of non- and low-sulfated GAG in 14 patients. It is suggested that the changes detected in the content and composition of GAG may contribute to platelet dysfunction in these diseases.

摘要

对24例副蛋白血症性成血细胞增多症患者(22例骨髓瘤病患者和2例瓦尔登斯特伦病患者)的血小板糖胺聚糖(GAG)含量及组成进行了研究。根据GAG水平,将患者分为两组:1)6例水平较高的患者(每100mg丙酮干燥细胞中含有181±8μg糖醛酸)和2)18例水平接近正常的患者(每100mg细胞中含有129±4μg)。第1组5例患者和第2组1例患者出现明显的出血素质表现。与健康人一样,硫酸软骨素在副蛋白血症性成血细胞增多症患者的血小板中占优势。14例患者因非硫酸化和低硫酸化GAG的次要成分数量减少而出现GAG电泳图谱缺失。提示在这些疾病中检测到的GAG含量及组成变化可能导致血小板功能障碍。

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