再生障碍性贫血出血机制临床探讨

Study on the Hemorragic Mechanism In Aplastic Anemia Patients

目的探讨血管因素、血小板及抗凝机制在再生障碍性贫血(再障)出血中的作用.方法采用ELISA法、火箭电泳法测定45例再障出血患者血浆中血栓调节蛋白(TM)、P-选择素、抗凝血酶Ⅲ(AT-Ⅲ)、血小板相关抗体(PAIgG)、D-二聚体等,与正常对照比较;对部分患者进行DIC相关指标测定;并对PAIgG与输血次数、血小板计数分别进行相关分析.结果9例急性再障患者血浆TM含量显著高于对照组,有8例伴发DIC及继发性纤溶亢进;45例再障(36例慢性再障和9例急性再障)血浆P-选择素增高同时伴有PAIgG增高;PAIgG增高与输血次数呈正相关(r=0.721,P＜0.01),与血小板数量呈负相关(r=-0.695,P＜0.01).再障时AT-Ⅲ活性高于正常对照组.结论再障患者血小板减少除因骨髓增生减少外,可能还有免疫机制参与.此外,再障患者AT-Ⅲ增高及血浆TM含量增高所致抗凝活性增强、凝血机制活性减弱及DIC等因素均可能参与再障的出血过程.

Objective To study the significance of blood vessels, platelets and anticoagulative mechanisms in the aplastic anemia (AA) patients with haemorrhagic tendency. Methods Fourty-five AA patients (9 acute and 36 chronic ones) were involved in the study. Thrombomodulin (TM) (a marker indicating vascular endothelium injury) and a kind of a-granule membrane protein (a molecular marker indicating platelet activity), were detected by ELISA. The content of AT-Ⅲ was measured by rocket electrophoresis. Results The plasma concentration of TM in the 9 acute AA patients was higher than that in the normal control group, and 8 of them had DIC and hyperfibrinolysis. The plasma concentrations of P-selectin and PAIgG were elevated in all of the 45 AA patients. The level of PAIgG was positively correlated to the transfusion (r=0.721,P<0.01) and negatively related to the platelet count (r=-0.695,P<0.01). The activity of AT-Ⅲ in the AA patients was higher than that in the controls. Conclusion The thrombocytopenia in AA patients is related to marrow hypocellularity and certain immune mechanisms. The increase of anticoagulative activities due to higher activity of AT-Ⅲ and higher plasma concentration of TM, as well as the lowered activity of coagulation and the occurrance of DIC, are all involved in the mechanism of haemorrhage.