遗传性球形红细胞增多症患儿的止血系统。
The hemostasis system in children with hereditary spherocytosis.
机构信息
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia; Center for Theoretical Problems of Physicochemical Pharmacology RAS, Moscow, Russia.
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
出版信息
Thromb Res. 2019 Apr;176:11-17. doi: 10.1016/j.thromres.2019.02.004. Epub 2019 Feb 5.
INTRODUCTION
Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises.
AIM
The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
METHODS
The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
RESULTS
TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p < 0.05 for TEG maximum amplitude; 28 ± 3 μm/min vs. 24 ± 2 μm/min, p < 0.05 for TD clot growth rate), while APTT, TT and PR were not significantly different between the two groups. Patients with HS were divided into 2 groups: those during hemolytic crisis (28 patients) and those without hemolytic crisis (34 patients). TEG and TD parameters were increased in those during hemolytic crisis compared to the steady state HS group (62 ± 5 mm vs. 57 ± 4 mm, p < 0.05 for TEG maximum amplitude; 31 ± 4 μm/min vs. 26 ± 3 μm/min, p < 0.05 for TD clot growth rate). The D-dimer levels were increased in 4 HS patients, for whom the activation of blood clotting was noted. Fibrinogen levels were decreased in patients with HS compared to the control group (2.1 ± 0.4 mg/ml vs. 2.6 ± 0.4 mg/ml, p < 0.05). Other tests were within the reference ranges for both groups.
CONCLUSIONS
The global hemostasis tests TEG and TD revealed hypercoagulability in patients with HS. More dramatic changes were observed in patients experiencing a hemolytic crisis.
简介
遗传性球形红细胞增多症（HS）患者的血栓形成风险增加。血栓并发症的早期表现甚至可在儿童期出现，尤其是在手术后。溶血性危象可伴有高凝状态。
目的
本研究旨在使用整体止血检测评估 HS 患儿的止血状态。
方法
使用凝血时间（APTT、TT 和 PR）、纤维蛋白原和 D-二聚体水平以及整体止血、血栓弹力图（TEG）和血栓动力学（TD）检测评估 62 名（38 名男孩和 24 名女孩；年龄范围：0.5 至 17 岁）HS 患儿在溶血危象期间和无溶血危象期间的止血状态。102 名接受年度体检的健康儿童作为对照组纳入研究。
结果
与对照组相比，HS 患儿的 TEG 和 TD 参数增加（TEG 最大振幅：60±5mm 比 53±4mm，p<0.05；TD 凝块生长速率：28±3μm/min 比 24±2μm/min，p<0.05），而 APTT、TT 和 PR 两组间无显著差异。HS 患儿分为两组：溶血危象组（28 例）和非溶血危象组（34 例）。与稳定期 HS 组相比，溶血危象组的 TEG 和 TD 参数增加（TEG 最大振幅：62±5mm 比 57±4mm，p<0.05；TD 凝块生长速率：31±4μm/min 比 26±3μm/min，p<0.05）。4 例 HS 患者的 D-二聚体水平升高，提示血液凝固激活。与对照组相比，HS 患儿的纤维蛋白原水平降低（2.1±0.4mg/ml 比 2.6±0.4mg/ml，p<0.05）。两组其他检测值均在参考范围内。
结论
整体止血检测 TEG 和 TD 显示 HS 患者存在高凝状态。在发生溶血危象的患者中观察到更显著的变化。
Zotero 插件