9 cases of von Willebrand's disease are reported to have various levels of factor VIII related activities. The assay for factor VIII coagulent (VIII: C) showed low levels but not less than 9% in 4 cases while those in the other 5 patients fell within the normal range (103±26%) or even elevated. The activities of factor VIII related antigen (VIII R: Ag) were ≤21% (3 cases) and between 29一57% (5 cases) respectively. One case with a high level of VIII R: Ag (116%) revealed a variant type demonstrated by its altered electrophoretic mobility on crossed immunoelectrophoretic assay. Estimations of factor VIII related Willebrand factor (VIII R: WF) were found decreased in 7 cases (≤3% in 3, between 26—60% in 4) and within the normal level (85±10%) in 2 cases.
Other data for hemostatic disorders included the followings: reduced platelet count (occasionally in I case), prolonged bleeding time (in 7/8), prolonged clotting time (in 1/5), prolonged KPTT (in 5/9), deficient platelet factor 3 availability (in 1/7) and diminished platelet adhesion to glass beads (in 1/6). Aspirin tolerance test performed in 4 cases on 11 occasions gave positive results in 7. 2 cases with deficient platelet aggregation to ADP (one with deficient platelet factor 3 availability) were suspected to have concurrent func tional disorders of platelets.
The present cases reported according to their clinical and laboratory findings can be subdivided into 3 categories: the classical form (3 cases), their variants (5cases), and carrier (1 case).