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血栓调节蛋白和血管性血友病因子:与儿童急性淋巴细胞白血病内皮功能障碍和疾病转归的关系。

Thrombomodulin and von Willebrand factor: relation to endothelial dysfunction and disease outcome in children with acute lymphoblastic leukemia.

机构信息

Department of Paediatrics, AHEPA Hospital, Thessaloniki, Greece.

出版信息

Acta Haematol. 2011;125(3):130-5. doi: 10.1159/000322120. Epub 2010 Dec 11.

DOI:10.1159/000322120
PMID:21150180
Abstract

The severe endothelial dysfunction in children with acute lymphoblastic leukemia (ALL) can result from the disease itself, from treatment, or from other conditions (e.g. sepsis). The aim of this study was to determine the levels of markers of endothelial activation in children with ALL and to assess their potential prognostic value. Fifty-two children with ALL, 19 children with ALL 1-10 years after the completion of therapy, and 28 healthy children were studied. In children with ALL, there was a significant increase in thrombomodulin (TM) and von Willebrand factor (vWF) levels during the acute phase of the disease and during treatment. Children with an unfavorable outcome had higher levels of TM. In conclusion, severe endothelial dysfunction is present during the acute phase of ALL and during treatment and appears to result from the disease itself. Serum TM and vWF levels might represent additional, but not independent, prognostic markers in childhood ALL.

摘要

儿童急性淋巴细胞白血病(ALL)可导致严重的内皮功能障碍,其病因可能来自疾病本身、治疗或其他情况(例如败血症)。本研究旨在确定 ALL 患儿内皮活化标志物的水平,并评估其潜在的预后价值。研究共纳入 52 例 ALL 患儿、19 例 ALL 患儿(完成治疗后 1-10 年)和 28 例健康儿童。在 ALL 患儿中,疾病急性期和治疗期间血栓调节蛋白(TM)和血管性血友病因子(vWF)水平显著升高。预后不良的患儿 TM 水平更高。综上,ALL 患儿在疾病急性期和治疗期间存在严重的内皮功能障碍,其病因可能来自疾病本身。血清 TM 和 vWF 水平可能是儿童 ALL 的附加但非独立的预后标志物。

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