Jonge Poerink-Stockschlader A B, Dekker I, Risseeuw-Appel I M, Hählen K
Department of Pediatrics, Division of Hematology/Oncology, Sophia Children's Hospital/University Hospital, Erasmus University, Rotterdam, The Netherlands.
Med Pediatr Oncol. 1996 Apr;26(4):238-43. doi: 10.1002/(SICI)1096-911X(199604)26:4<238::AID-MPO3>3.0.CO;2-K.
The association between acquired von Willebrand disease and Wilms' tumor has been reported in eight cases: four cases reports and one prospective study of Coppes et al. (J. Clin Oncol 10:422-427, 1992) who found this in four out of 50 patients. We retrospectively studied 73 children who were diagnosed with a Wilms' tumor between 1970 and 1993. All patients were treated according to the running international SIOP protocol. According to our local diagnostic workup protocol, blood samples for screening coagulation tests were obtained at diagnosis and during preoperative chemotherapy. Since 1984, factor VIII analysis was added. In four patients, no coagulation screen was done. Bleeding time and screening tests apart from APTT were normal in all 69 children tested before or within 2 days after starting therapy. In 47 out of 73 patients, an APTT was performed before starting therapy. In 19 patients (40%), it was prolonged (>33 sec). In 8 of them (17%), the prolongation was severe (> or = 40 sec). In 11 out of the 19 patients, factor VIIIc, factor VIIIag, and factor VIII RcoF determinations were done. In two children, all three factors were decreased suggestive for von Willebrand disease. One of the 19 patients with a prolonged APTT had hematuria. The others had no increased bleeding tendency or signs of bleeding in the tumor. In all patients, the prolonged APTT normalised during preoperative chemotherapy within 6 weeks. Frequent blood samples were obtained of the two children with acquired von Willebrand disease and showed normalisation of the coagulation disorder after 1 and 2 weeks, respectively. No specific therapy to correct the coagulation abnormalities was given to any patient.
获得性血管性血友病与肾母细胞瘤之间的关联已有8例报道：4例病例报告以及Coppes等人的一项前瞻性研究（《临床肿瘤学杂志》10:422 - 427, 1992），后者在50例患者中发现了4例。我们回顾性研究了1970年至1993年间被诊断为肾母细胞瘤的73名儿童。所有患者均按照当时正在执行的国际小儿肿瘤学会（SIOP）方案进行治疗。根据我们当地的诊断检查方案，在诊断时以及术前化疗期间采集血液样本进行凝血筛查试验。自1984年起，增加了因子VIII分析。4例患者未进行凝血筛查。在开始治疗前或治疗后2天内接受检测的所有69名儿童中，除活化部分凝血活酶时间（APTT）外，出血时间及其他筛查试验均正常。73例患者中有47例在开始治疗前进行了APTT检测。其中19例患者（40%）APTT延长（>33秒）。其中8例患者（17%）延长严重（≥40秒）。19例APTT延长的患者中有11例进行了因子VIIIc、因子VIII抗原（VIIIag）和因子VIII相关抗原（RcoF）测定。2名儿童的所有三项因子均降低，提示患有血管性血友病。19例APTT延长的患者中有1例出现血尿。其他患者无出血倾向增加或肿瘤出血迹象。所有患者延长的APTT在术前化疗6周内恢复正常。对2例获得性血管性血友病儿童频繁采集血样，结果显示凝血障碍分别在1周和2周后恢复正常。未对任何患者给予纠正凝血异常的特异性治疗。
