Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH
Factor IX-Inhibitors
Report of Two Cases and a Study of the Biological, Chemical and Immunological Properties of the Inhibitors
K Lechner
1
First Department of Medicine, University of Vienna (Head: Prof. Dr. E. Deutsch)
› Author AffiliationsThis work has been supported by a grant from the “Österreichische Fonds zur Förderung der wissenschaftlichen Forschung” Nr. 703 and 900.
1. Two patients with severe hemophilia B and an acquired inhibitor against factor IX are described.
2. The inhibitors inactivate factor IX irreversibly. The speed of inactivation is very fast and cannot slowed down by dilution of the inhibitor or by lowering of the incubation temperature. Factor IX of serum is inactivated to a higher degree than factor IX of plasma. Factor IX inhibitors are more sensitive to dilution than factor VIII inhibitors.
3. The inhibitor protein is eluted from the Sephadex G-200 column with the G-peak. On Chromatography on DEAE-cellulose two activity peaks are obtained.
4. Immunologically the inhibitors belong predominantly to the immune globulins of the G-class, but it cannot be excluded that the activity is also associated with IgA. As light chains kappa chains were found in one case.
References
1
Andersen B. R,
Trowp S. B.
Gamma G-antibody to human antihemophilic globulin (factor VIII). J. Immunol 100: 175 1968;
5
Fantl P,
Sawers R. J,
Marr A. G.
Investigation of a hemorrhagic disease due to beta-prothromboplastin deficiency complicated by a specific inhibitor of thromboplastin formation. Aust. Ann. Med 05: 163 1956;
6
Goldstein R,
Gelfand M,
Sanders M,
Rosen R.
Anticoagulant appearing in plasma thromboplastin component (PTC) deficiency (Abstr.). J. clin. Invest 35: 707 1956;
9
Lechner K.
Inhibitors in iso- and autoimmune disease. I. Conf. Thrombosis and Hemostasis (Montreux) 1970. Thrombos. Diathes. haemorrh. (Stuttg). 1971 (In press.)
11
Lewis J. H,
Ferguson J. H,
Arends T.
Hemorrhagic disease with circulating inhibitors of blood clotting: anti-AHF and anti-PTC in eight cases. Blood 11: 846 1956;
12
Margolius A,
Jackson D. P,
Ratnoff O. D.
Circulating anticoagulants: a study of 40 cases and a review of the literature. Medicine (Baltimore.) 40: 145 1961;
14
Roberts H. R,
Gross G. P,
Webster W. P,
Dejanow I. I,
Penick G. D.
Acquired inhibitors to plasma factor IX: A study of their induction, properties and neutralisation. Amer. J. med. Sci 251: 43 1960;
15
Shajpiro S. S.
The immunological character of acquired inhibitors of antihemophilic globulin (F VIII) and the kinetics of their interaction with factor VIII. J. clin. Invest 46: 147 1967;
