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携带MTHFR TT677基因型及其他血栓形成风险因素的急性淋巴细胞白血病儿童的脑静脉窦血栓形成

Cerebral venous sinus thrombosis in children with acute lymphoblastic leukemia carrying the MTHFR TT677 genotype and further prothrombotic risk factors.

作者信息

Wermes C, Fleischhack G, Junker R, Schobess R, Schwabe D, Sykora K W, Nowak-Göttl U

机构信息

Pediatric Hematology/Oncology, University Hospitals Bonn, Germany.

出版信息

Klin Padiatr. 1999 Jul-Aug;211(4):211-4. doi: 10.1055/s-2008-1043790.

DOI:10.1055/s-2008-1043790
PMID:10472552
Abstract

BACKGROUND

The present study was designed to prospectively evaluate the role of prothrombotic risk factors in leukemic children treated according to the ALL-BFM 90/95 study protocols with respect to the onset of cerebral venous sinus thrombosis.

PATIENTS

317 consecutive leukemic children aged 6 months to 18 years were enrolled in this study. 288 of the 317 patients were available for thrombosis-free survival analysis.

RESULTS

In 17 (5.9%) of these 288 patients cerebral venous sinus thrombosis occurred. The overall event-free survival of thrombosis in the central nervous system in patients with at least one prothrombotic defect (n = 12) was significantly reduced compared with patients without a prothrombotic defect (p < 0.0001). 15 patients showed acute clinical symptoms at onset of cerebral venous sinus thrombosis, two were asymptomatic. Three of the 17 patients affected (17.6%) died directly associated with the thrombotic event during induction therapy, the remaining 14 patients did not show prolonged clinical symptoms.

CONCLUSIONS

Prothrombotic risk factors should be included in a screening program in ALL children treated according to the BFM study protocols. Further prospective studies are recommended to establish adequate prophylactic anticoagulant treatment during ALL (BFM) polychemotherapy.

摘要

背景

本研究旨在前瞻性评估根据ALL - BFM 90/95研究方案治疗的白血病儿童中,血栓形成前危险因素在脑静脉窦血栓形成发病方面的作用。

患者

本研究纳入了317例年龄在6个月至18岁之间的连续性白血病儿童。317例患者中有288例可进行无血栓生存分析。

结果

在这288例患者中,有17例(5.9%)发生了脑静脉窦血栓形成。至少有一项血栓形成前缺陷的患者(n = 12)中枢神经系统血栓形成的总体无事件生存率与无血栓形成前缺陷的患者相比显著降低(p < 0.0001)。15例患者在脑静脉窦血栓形成发作时出现急性临床症状,2例无症状。17例受影响患者中有3例(17.6%)在诱导治疗期间直接死于血栓形成事件,其余14例患者未出现长期临床症状。

结论

在根据BFM研究方案治疗的所有白血病儿童的筛查项目中应纳入血栓形成前危险因素。建议进一步进行前瞻性研究,以确定在ALL(BFM)多药化疗期间适当的预防性抗凝治疗。

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