Immunologie Studies in von Willebrand’s Disease^[*]

Summary

Two patients with a severe von Willebrand’s disease characterized by no detectable factor VIII related antigen in their plasma received transfusions of cryoprecipitate. The bleeding time was corrected for a short period of time and returned to its pretransfusional value although the other parameters of the disease were still corrected. Electrophoretic and immunologic properties of factor VIII related antigen infused were determined serially after transfusion. Modifications of these properties occurred progressively after transfusion. The half disappearance time of F. VIIIR. A. was determined and found to be considerably shorter than in hemophilic recipients. This study suggests an alteration in vivo of F. VIIIR. A. infused into von Willebrand recipients.

^* Presented at the Vth Congress of the International Society on Thrombosis and Haemostasis, Paris, July, 1975.

• Reference

• 1 Bennett B, Ratnoff O. 1972; Studies on the response of patients with classic hemophilia to transfusion with concentrates of antihemophilic factor. A difference in the half life of antihemophilic factor as measured by procoagulant activity and immunologic techniques. Journal of Clinical Investigation 51: 2593.
• 2 Bennett B, Ratnoff O, Levine J. 1972; Immunologic studies in von Willebrand’s disease. Evidence that the antihemophilic factor (AHF) produced after transfusion lacks an antigen associated with normal AHF and the inactive material produced by patients with classic hemophilia. Journal of Clinical Investigation 51: 2597.
• 3 Bloom A. L, Peake I. R, Giddings J. C. 1973; The presence and reactions of high and lower molecular weight procoagulant factor VIII in the plasma of patients with von Willebrand’s disease after treatment: significance for a structural hypothesis for factor VIII. Thrombosis Research 3: 389.
• 4 Borchgrevink C. F. 1960; A method for measuring platelet adhesiveness in vivo. Acta Medica Scandinavica 168: 157.
• 5 Cornu P, Larrieu M.-J, Caen J, Bernard J. 1963; Transfusion studies in von Willebrand’s disease: effect on bleeding time and factor VTII. British Journal of Haematology 9: 189.
• 6 Holmberg L, Mannucci P. M, Turesson I, Ruggeri Z. M, Nilsson I. M. 1974; Factor VIII antigen in the vessel walls in von Willebrand’s disease and haemophilia A. Scandinavian Journal of Haematology 13: 33.
• 7 Hoyer L. W, De Los Santos R. P, Hoyer J. R. 1973; Antihemophilic factor antigen: localization in endothelial cells by immunofluorescent microscopy. Journal of Clinical Investigation 52: 2737.
• 8 Jaffe E. A, Hoyer L. W, Nachman R. L. 1974; Synthesis of antihemophilic factor antigen by cultured human endothelial cells. Journal of Clinical Investigation 52: 2757.
• 9 Laurell C. B. 1965; a Antigen antibody crossed electrophoresis. Analytical Biochemistry 10: 358.
• 10 Laurell C. B. 1966; b Electrophoretic heterogeneity alpha₂ macroglobulin. Clinica Chimica Acta 14: 137.
• 11 Nilsson I. M, Blomback M, Blomback B. 1959; von Willebrand’s disease in Sweden. Its pathogenesis and treatment. Acta Medica Scandinavica 164: 263.
• 12 Ratnoff O. D, Saito H. 1974; Bleeding in von Willebrand’s disease. New England Journal of Medicine 290: 1089.
• 13 Soulier J. P, Larrieu M.-J. 1953; Measurement of thromboplastic factors and profactors in plasma. I. Deficits in thromboplastin: study of reagents measurement of antihemophilic and of platelet activities. Journal of Laboratory and Clinical Medicine 41: 849.
• 14 Sultan Y, Simeon J, Caen J. P. 1975 a Electrophoretic heterogeneity of normal factor VIII/ von Willebrand protein, and abnormal electrophoretic mobility in patients with von Willebrand’s disease. Journal of Laboratory and Clinical Medicine. (In press).
• 15 Sultan Y, Simeon J, Caen J. P. 1975; b Detection of heterozygotes in both parents of homozygous patients with von Willebrand’s disease. Journal of Clinical Pathology 28: 309.
• 16 Thomson C, Forbes C. D, Prentice C. R. M. 1974; Evidence for a qualitative defect in F. VIII related antigen in von Willebrand’s disease. Lancet I: 594.
• 17 Veltkamp J. J, van Tilburg N. H. 1973; Detection of heterozygotes for recessive von Willebrand’s disease by the assay of antihemophilic-factor-like antigen. New England Journal of Medicine 289: 882.
• 18 Weiss H. J, Hoyer L. W, Rickles F. R, Varna A, Rogers J. 1973; Quantitative assay of a plasma factor deficient in von Willebrand’s disease that is necessary for platelet aggregation. Relationship to factor VIII procoagulant activity and antigen content. Journal of Clinical Investigation 52: 2708.
• 19 Zimmerman T. S, Ratnoff O. D, Powell A. E. 1971; Immunologic differentiation of classic hemophilia (factor VIII deficiency and von Willebrand’s disease). Journal of Clinical Investigation 50: 244.