Clinical Significance of Inhibitors in Acquired von Willebrand Syndrome

摘要: Of 260 patients enrolled, 25 (9.6%) were associated with acquired von Willebrand syndrome (AvWS). We studied AvWS, retrospectively. AvWS was diagnosed by reduced levels of factor (vWF) (decrease antigen [vWF:Ag] and ristocetin cofactor [vWF:RCoF]), a decrease ristocetin-induced platelet agglutination (RIPA), sometimes decreased high-molecular-weight multimers, prolonged bleeding time neither prior nor family histories problems the evidence normal vWF:RCoF in their families. The inhibitor vWF determined mixing patient plasma pooled plasma. Eight this study had inhibitors to that IgG class; subclasses IgG1 (7 cases) IgG2 (1 case). Multimeric analysis showed selective loss large multimers most similar congenital type-2 disease (vWD). All blocked ristocetin-mediated binding platelets. Five out 6 IgGs evaluated here recognized 39/34-kD fragment (residues 480/481-718) Fragment III 1-1365) implied domain glycoprotein Ib (GPIb), whereas 1 I 911-1365). A close relationship found between presence tendency. 7 inhibitors, (86%) tendency, as well 15 without (6%). efficacy treatment underlying diseases and/or therapy deamino D-arginine vasopressin (DDAVP) for also depends on an inhibitor. Four 8 (50%) poor response DDAVP, 16 These results indicate developing are likely have might be resistant DDAVP AvWS. intravenous immunoglobulin (0.3 g/kg, 3 days) effective correct hemostatic defect manage severe inhibitors. consider additional including expensive high-dose when uncontrollable is continued after DDAVP.