Transplantation of normal bone marrow into a pig with severe von Willebrand's disease.
Bone marrow from a normal male pig was transplanted into a related female pig with severe homozygous von Willebrand's disease (vWd). After engraftment the circulating leukocytes were of the male karyotype, and the platelets were strongly positive for von Willebrand factor (vWF) by indirect immunofluorescence. The average level of vWF was 1.96 U/dl and of ristocetin cofactor was 2.8 U/dl. The ear immersion bleeding time before transplantation was consistently more than 15 min and afterwards varied between 5 min and more than 15 min. Transfused vWF corrected the bleeding time at a level of 10 U/dl, which is lower than that required for a von Willebrand pig. We concluded that: the plasmatic compartment is only minimally replenished by the vWF from platelets and megakaryocytes; and the platelet vWF alone only partially corrects the abnormal tests of the hemostatic mechanism in severe vWd.
D000818:Animals; D000941:Antigens / Q000032:analysis; D001760:Bleeding Time; D001792:Blood Platelets / Q000032:analysis; D001803:Blood Transfusion; D016026:Bone Marrow Transplantation; D003894:Deamino Arginine Vasopressin / Q000494:pharmacology; D004591:Electrophoresis, Polyacrylamide Gel; D005169:Factor VIII / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D006487:Hemostasis; D006720:Homozygote; D007621:Karyotyping; D007962:Leukocytes / Q000032:analysis; D008297:Male; D013552:Swine; D014184:Transplantation, Homologous; D014842:von Willebrand Diseases / Q000235:genetics / Q000628:therapy*; D014841:von Willebrand Factor / Q000032:analysis
D000941:Antigens; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin