Abstract
Antihemophilic globulin (AHF, factor VIII) levels were measured by a standard coagulation assay and by an immunological technique before and serially after infusion of fresh frozen plasma or cryoprecipitate into patients with von Willebrand's disease. Initial levels of AHF, measured both as procoagulant and as antigen, were low. Immediately after transfusions, the rise in levels of AHF-like antigen was compatible with the quantity of antigen present in the infused plasma or cryoprecipitate. Thereafter, levels of antigen declined rapidly and reached preinfusion values in approximately 24 hr. In contrast, procoagulant activity remained elevated, and sometimes continued to rise, for longer periods of time. One possible explanation of this finding is that the AHF molecule produced by patients with von Willebrand's disease, in response to transfusion of as yet unidentified factors, lacks the antigenic site associated with the normal AHF molecule or the inactive molecule produced by patients with hemophilia A.
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Selected References
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- Bennett B., Ratnoff O. D. 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 and immunologic techniques. J Clin Invest. 1972 Oct;51(10):2593–2596. doi: 10.1172/JCI107076. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Bennett E., Huehns E. R. Immunological differentiation of three types of haemophilia and identification of some female carriers. Lancet. 1970 Nov 7;2(7680):956–958. doi: 10.1016/s0140-6736(70)92129-x. [DOI] [PubMed] [Google Scholar]
- Bouma B. N., Wiegerinck Y., Sixma J. J., Van Mourik J. A., Mochtar I. A. Immunological characterization of purified anti-haemophilic factor A (factor VIII) which corrects abnormal platelet retention in Von Willebrand's disease. Nat New Biol. 1972 Mar 29;236(65):104–106. doi: 10.1038/newbio236104a0. [DOI] [PubMed] [Google Scholar]
- CORNU P., LARRIEU M. J., CAEN J., BERNARD J. Transfusion studies in von Willebrand's disease: effect on bleeding time and factor VIII. Br J Haematol. 1963 Apr;9:189–202. doi: 10.1111/j.1365-2141.1963.tb05457.x. [DOI] [PubMed] [Google Scholar]
- Denson K. W., Biggs R., Haddon M. E., Borrett R., Cobb K. Two types of haemophilia (A+ and A-): a study of 48 cases. Br J Haematol. 1969 Aug;17(2):163–171. doi: 10.1111/j.1365-2141.1969.tb01355.x. [DOI] [PubMed] [Google Scholar]
- GOUDEMAND M., FOUCAUT M., HUTIN A., PARQUET-GERNEZ A. LES ANTICOAGULANTS CIRCULANTS ANTI-FACTEUR VIII AU COURS DE L'H'EMOPHILIE A (NATURE ET M'ECANISME D'ACTION.) Nouv Rev Fr Hematol. 1963 Nov-Dec;3:703–722. [PubMed] [Google Scholar]
- Hoyer L. W. Immunologic studies of antihemophilic factor (AHF, factor VIII). 3. Comparative binding properties of human and rabbit anti-AHF. Blood. 1972 Apr;39(4):481–489. [PubMed] [Google Scholar]
- NILSSON I. M., BLOMBACK M., BLOMBACK B. v. Willebrand's disease in Sweden; its pathogenesis and treatment. Acta Med Scand. 1959 Jun 30;164:263–278. [PubMed] [Google Scholar]
- Rizza C. R., Eipe J. Exercise, factor VIII and the spleen. Br J Haematol. 1971 Jun;20(6):629–635. doi: 10.1111/j.1365-2141.1971.tb00801.x. [DOI] [PubMed] [Google Scholar]
- Salzman E. W. Measurement of platelet adhesiveness: progress report. Thromb Diath Haemorrh Suppl. 1967;26:303–307. [PubMed] [Google Scholar]
- Stites D. P., Hershgold E. J., Perlman J. D., Fudenberg H. H. Factor 8 detection by hemagglutination inhibition: hemophilia A and von Willebrand's disease. Science. 1971 Jan 15;171(3967):196–197. doi: 10.1126/science.171.3967.196. [DOI] [PubMed] [Google Scholar]
- Uszyński L. The immunological properties of factor VIII. II. The immunoelectrophoretic studies. Thromb Diath Haemorrh. 1967 Dec 31;18(3-4):325–331. [PubMed] [Google Scholar]
- Zimmerman T. S., Ratnoff O. D., Powell A. E. Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand's dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest. 1971 Jan;50(1):244–254. doi: 10.1172/JCI106480. [DOI] [PMC free article] [PubMed] [Google Scholar]