Thrombosis and Haemostasis, Table of Contents Thromb Haemost 1968; 20(01/02): 144-154DOI: 10.1055/s-0038-1651255 Originalarbeiten – Original Articles – Travaux Originaux Schattauer GmbH A Circulating Anticoagulant Directed against Antihaemophilic Globulin (Factor VIII) Combined with Thrombocytopenia Report of a Case Successfully Treated by an Immunosuppressive Agent[*] G Winckelmann 1 Department of Medicine (Commiss. Director: Prof. Dr. J. Schirmeister), University of Freiburg, Germany , D Böttcher 1 Department of Medicine (Commiss. Director: Prof. Dr. J. Schirmeister), University of Freiburg, Germany , R Groh 1 Department of Medicine (Commiss. Director: Prof. Dr. J. Schirmeister), University of Freiburg, Germany , with Technical Assistance by M. Rehe › Author Affiliations Recommend Article Abstract PDF Download Buy Article Summary A circulating anticoagulant directed against factor VIII (antihaemophilic globulin) is described which developed in association with thrombocytopenia in an otherwise apparently healthy woman. Inactivation of factor VIII by this inhibitor was shown to be progressive and temperature dependent. The circulating anticoagulant was not species specific and produced temporary factor VIII deficiency when injected into dogs. Administration of large doses of corticosteroids to the patient did not affect the factor VIII inhibitor and resulted only in a temporary increase in the platelet counts. In the course of subsequent immunosuppressive treatment with azathioprine, the inhibitor disappeared and clinical symptoms improved, whereas thrombocytopenia persisted. PDF (1807 kb) References References 1 Andersen B.R, Terry W.D. Gamma G4-globulin antibody causing inhibition of clotting factor VIII. Nature (Lond.) 1968; 217: 174-175 2 Bachmann F, Duckert F, Koller F. The Stuart-Prower factor assay and its clinical significance. Thrombos. Diathes. haemorrh. (Stuttg.) 1958; 2: 24-38 3 Barkhan P. Observations on a coagulation defect characterized by thrombocytopenia with a circulating anticoagulant. S. Afr. J. med. Sci 1952; 17: 87-89 4 Benthaus J. Über die Retraktion des Blutgerinnsels. Thrombos. Diathes. haemorrh. (Stuttg.) 1959; 5: 311-347 5 Bidwell E, Benson K.W.E, Dike G.W.R, Augustin R, Lloyd G.M. Antibody nature of the inhibitor to antihaemophilic globulin (factor VIII). Nature (Lond.) 1966; 210: 746-747 6 Biggs R, Douglas A.S. The thromboplastin generation test. J. clin. Path 1953; 6: 23-29 7 Biggs R, Bidwell E. A method for the study of antihaemophilic globulin inhibitors with reference to six cases. Brit. J. Haemat 1959; 5: 379-395 8 Biggs R, Denson K.W.E, Nossel H.L. A patient with an unusual circulating anticoagulant. Thrombos. Diathes. haemorrh. (Stuttg.) 1964; 12: 1-11 9 Bläker F, Fischer K, Landbeck G. Entwicklung von Hemmkörpern nach langfristiger Substitution von humanem antihämophilen Globulin bei schwerer Hämophilie A. Klin. Wschr 1967; 45: 630-633 10 Bonnin J.A, Cohen A.K, Hicks N.D. Coagulation defects in a case of systemic lupus erythematosus with thrombocytopenia. Brit. J. Haemat 1956; 2: 168-179 11 Borchgrevink C.F. A method for measuring platelet adhesiveness in vivo. Acta med. scand 1960; 168: 157-164 12 Borchgrevink C.F, Waaler B.A. The secondary bleeding time. A new method for the differentiation of hemorrhagic diseases. Acta med. scand 1958; 162: 362-374 13 Breckenridge R.T, Ratnoff O.D. Studies on the nature of the circulating anticoagulant directed against antihemophilic factor: with notes on an assay for antihemophilic factor. Blood 1962; 20: 137-149 14 Van Grefeld S, Hoorweg P.G, Paulssen M.M.P. Researches on a circulating anticoagulant in a hemophiliac, II. Effect of administration of ACTH and cortisone. Blood 1953; 8: 125-130 15 Dausset J. Les anticoagulants circulants sontils des anticorps?. Sang 1959; 30: 428-441 16 Deutsch E. Acquired inhibitors. Thrombos. Diathes. haemorrh. (Stuttg.) 1962; 7: 112-128 Suppl. 1 ad 17 Deutsch E, Frischauf H, Keibl E. Hemmkörperhämophilie als Komplikation bei essentieller Thrombopenie. Acta haemat. (Basel) 1951; 6: 156-173 18 Ehrenworth L. Spontaneously occurring anticoagulant against antihemophilic globulin in a previously normal subject. Amer. J. Med 1963; 34: 272-280 19 Favry-Gilly J, Thouverez J.P. Les inhibiteurs de la première phase de la coagulation en dehors de l’hémophilie. Sang 1959; 30: 351-376 20 Feissly R, Lüdin H. Microscopie par contrastes de phases, III. Applications à l’hématologie. Rev. Hémat 1949; 4: 481-501 21 Goudemand M.L, Foucaut M, Hutin A, Parquet-Gernez A. Les anticoagulants circulants anti-facteur VIII au cours de l’hémophilie A (nature et méchanisme d’action). Nouv. Rev. franç, hémat 1963; 3: 703-722 22 Hartert H. Blutgerinnungsstudien mit der Thrombelastographie, einem neuen Untersuchungsverfahren. Klin. Wschr 1948; 26: 577-583 23 Horowitz H.I, Fujimoto M.M. Acquired hemophilia due to a circulating anticoagulant. Report of two cases, with review of the literature. Amer. J. Med 1962; 33: 501-509 24 Lee S.L, Sanders M. A disorder of blood coagulation in systemic lupus erythematosus. J. clin. Invest 1955; 34: 1814-1822 25 Lopaciuk S, Naleczynska A, Czaja H, Walewska I, Pawelski S. Circulating anticoagulant against factor VIII coexistent with some immunological reactions in a female. Thrombos. Diathes. haemorrh. (Stuttg.) 1964; 11: 444-451 26 Margolius Jr. A, Jackson D.P, Ratnoff O.D. Circulating anticoagulants: a study of 40 cases and a review of the literature. Medicine (Baltimore) 1961; 40: 145-202 27 Masure R. Les inhibiteurs normaux et pathologiques de la coagulation sanguine. Edition Arscia; Bruxelles: 1960 28 Mayer G, Mayer S, Waitz R. Anticoagulant circulant associé à une thrombopénie. Hémostase 1965; 5: 21-36 29 Medal L.S, Lisker R. Circulating anticoagulants in disseminated lupus erythematosus. Brit. J. Haemat 1959; 5: 284-293 30 Nilsson I.M, Skanse B, Gydell K. Circulating anticoagulant after pregnancy and its response to ACTH. Acta haemat. (Basel) 1958; 19: 40-50 31 Pinkerton P.H, Dagg J.H, Taylor F. A circulating anticoagulant inhibiting antihaemophilic globulin. J. clin. Path 1965; 18: 334-338 32 Pitney W.R. The assay of antihaemophilic globulin (AHG) in plasma. Brit. J. Haemat 1956; 2: 250-264 33 Roberts H.R, Scales M.B, Madison J.T, Webster W.P, Penick G.D. A clinical and experimental study of acquired inhibitors to factor VIII. Blood 1965; 26: 805-818 34 Rodman Jr. N.F, Barrow E.M, Graham J.B. Diagnosis and control of hemophiloid states with the partial thromboplastin time (PTT) test. Amer. J. clin. Path 1958; 29: 525-538 35 Shapiro S.S. The immunologic character of acquired inhibitors of antihemophilic globulin (factor VIII) and the kinetics of their interaction with factor VIII. J. clin. Invest 1967; 46: 147-156 36 Strauss H.S, Merler E. Characterization and properties of an inhibitor of factor VIII in the plasma of patients with hemophilia A following repeated transfusions. Blood 1967; 30: 137-150 37 Whitfield A.G.W, Meynell M.J, Fessey B.M. A circulating anticoagulant occurring after temporal arteritis and controlled by corticosteroid therapy. J. clin. Path 1962; 15: 357-360 38 Winckelmann G, Overbeck W. Blutgerinnungsprobleme beim extrakorporalen Kreislauf. Klin. Wschr 1961; 39: 333-338 39 Winterstein A, Marbet R, Strässle R. Kritische Betrachtung zum Thromboplastin-Ge- neration-Test. (Abstr.) Proc. 5th Congr. Europ. Soc. Haemat., Freiburg 1955. Berlin-Göttingen-Heidelberg; Springer: 1956: 803