Replacement therapy in platelet-type von Willebrand disease.
The response to infusions of cryoprecipitate and factor VIII concentrate was studied in a patient with platelet-type von Willebrand disease (vWD) who showed lack of the large multimers of von Willebrand factor in plasma, increased platelet aggregation with low concentrations of ristocetin, and in vitro platelet aggregation by normal plasma. The cryoprecipitate and factor VIII concentrate to be infused induced platelet aggregation when added to patient platelet-rich plasma at concentrations higher than 0.86 U/ml and 3 U/ml of factor VIII-related antigen (VIIIR:Ag), respectively. Administration of cryoprecipitate (41.9 U VIIIR:Ag/kg body weight) was followed by a shortening of the bleeding time, and hemostasis was achieved during tooth extractions. Factor VIII concentrate (70.2 U VIIIR:Ag/kg) failed to correct the prolonged bleeding time and proved ineffective to control the gum bleeding. No significant diminution of the platelet count was observed following any infusion. These results indicate that cryoprecipitate is hemostatically effective and safe when infused in such a dosage, but factor VIII concentrate is not effective in platelet-type vWD in analogy to what is observed in various types of vWD.
D000941:Antigens / Q000627:therapeutic use*; D001760:Bleeding Time; D001777:Blood Coagulation / Q000187:drug effects; D002648:Child; D003450:Cryoglobulins / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology* / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D012310:Ristocetin / Q000494:pharmacology; D014081:Tooth Extraction; D014842:von Willebrand Diseases / Q000097:blood / Q000628:therapy*; D014841:von Willebrand Factor / Q000032:analysis
D000941:Antigens; D003450:Cryoglobulins; D014841:von Willebrand Factor; D012310:Ristocetin; D005169:Factor VIII