von Willebrand disease in a pediatric-based population--comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor/collagen-binding assay.

摘要: Definitive diagnosis of type 1 von Willebrand Disease (VWD) remains a problem. Provisional consensus guidelines for the definite and possible VWD were prepared by Scientific Subcommittee on factor (VWF) Standardization Committee (SSC) International Society Thrombosis Haemostasis (ISTH) during 1996 annual meeting specific purpose further evaluation in retrospective prospective studies Working Party Diagnostic Criteria (1996 Annual Report SSC/ISTH VWF). In first phase this study, we compared 2 definitions VWD. each with 3 criteria: significant bleeding history, laboratory investigations, family history. Using ISTH definition, significantly fewer patients diagnosed disease as to our "in house" Hospital Sick Children (HSC) criteria (4 vs. 31). While recognize that provisional not intended clinical use, believe results are interest will assist any future refinements guidelines. second investigated utility new tests, screening test functional test, well characterized, pediatric-based population. The Platelet Function Analyzer (PFA-100) provides an vitro measure primary hemostasis under conditions high shear, using disposable cartridges containing collagen either epinephrine or ADP. All tested subjects types had prolonged PFA-100 closure times (CTs) both cartridge (n = 17) 14). VWD, 20/24 (83%) CTs collagen/ADP (19/24 (79%) collagen/epinephrine), 7/26 (27%) times. 1, 2, abnormal 37/41 subjects, giving overall sensitivity 90%. With sensitivity, is better than time. We also VWF collagen-binding assay (VWF:CBA) VWF, comparison more routinely-used ristocetin cofactor (VWF:RC0). VWF:CBA based ELISA technique, which has potential be reproducible VWF:RC0. found detected 43/49 (88%) performing VWF:RC0, 42/48 (88%). showed that, used conjunction antigen levels, may useful classification subtypes.