ACQUIRED VON-WILLEBRAND DISEASE IN CHILDREN WITH A WILMS-TUMOR

The association between acquired von Willebrand disease and Wilms' tum or has been reported in eight cases: four case reports and one prospec tive 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 childr en who were diagnosed with a Wilms' tumor between 1970 and 1993. All p atients were treated according to the running international SIOP proto col. 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 s creening tests apart from APTT we re normal in all 69 children tested before or within 2 days after starting therapy. In 47 out of 73 patien ts, an APTT was performed before starting therapy. In 19 patients (40% ), it was prolonged (>33 sec). In 8 of them (17%), the prolongation wa s severe (greater than or equal to 40 sec). In 11 out of the 19 patien ts, factor VIIIc, factor VIIIag, and factor VIII RcoF determinations w ere done. In two children, all three factors were decreased suggestive for von Willebrand disease. One of the 19 patients with a prolonged A PTT had hematuria. The others had no increased bleeding tendency or si gns of bleeding in the tumor. In all patients, the prolonged APTT norm alised during preoperative chemotherapy within 6 weeks. Frequent blood samples were obtained of the two children with acquired von Willebran d disease and showed normalisation of the coagulation disorder after 1 and 2 weeks, respectively. No specific therapy to correct the coagula tion abnormalities was given to any patient. (C) 1996 Wiley-Liss, Inc.