红细胞收缩:血小板无力症的可能代偿机制?  

RED BLOOD CELL CLOT RETRACTION: POSSIBLE COMPENSATIVE MECHANISM FOR PATIENTS WITH GLANZMANN THROMBASTHENIA?

作　　者：解勤之[1] 陈方平[1] 蹇在伏[1] 赵谢兰[1] 谭柏林[1] 

机构地区：[1]湖南医科大学湘雅医院血液科

出　　处：《中国现代医学杂志》1998年第11期3-4,共2页China Journal of Modern Medicine

基　　金：湖南省自然科学基金

摘　　要：该文对２例Ⅰ型血小板无力症进行了１３年的临床观察。发现患者出血症状随年龄增长明显减轻，全血血块回缩（ＣＲ）也由初诊时的不良转变为良好。进一步研究发现：患者血小板ＣＲ仍有缺陷，Ｍｇ２＋对血小板ＣＲ缺陷无改善作用，但患者红细胞ＣＲ（分别为０．５２和０．６０）较正常（０．４０）好，将洗涤后的正常“Ｏ”型血红细胞分别悬浮在患者和正常新鲜血浆中所作的ＣＲ二者无区别，该研究表明：患者红细胞而不是血浆因素（纤维蛋白质）可能发挥了部份代偿全血ＣＲ的作用。Two type female patients with Glanzmann thrombasthenia (GT) were carried out follow up study for 13 years,which interestinaly showed their first CR 13 years ago.Further study revealed both patients bleeding symptoms improved with their ages up and their clot retraction (CR) turned to be perfect compared with their first CR 13 years ago.Further study revealed both patientsplatelet CR remained impaired which could not be improved by Mg 2+ , but patients red blood cell (RBC) CR (52% and 60% respectively) retracted better than normal controls (40%). However,there was no significant difference between patients and controls in RBC CR when normal “0” type donor RBC was washed and suspended in fresh plasmas from patients and controls. The result suggest that patients RBC other than plasma(fibrinognen) might play a role in compensation for these 2 GT patients.

关 键 词：血小板无力症 血块回缩 病理 

分 类 号：R558.02[医药卫生—血液循环系统疾病]