Failure of Immunosuppression in a Severe Haemophilia B Patient with Specific Antibody

Summary

Prevention of a secondary response to factor IX by cyclophosphamide was attempted in an 11 year old patient with severe Christmas disease. An antibody to factor IX had been present for 4 years before immunosuppressive therapy was tried. Despite profound lymphopenia, synthesis of factor IX antibody was not depressed. The difficulties of modifying the anamnestic response to factor IX by chemical immunosuppression may be as real as has been reported for factor VIII in classical haemophilia.

• References

• 1 Allain J. P, Frommel D. 1973; Antibodies to Factor VIII: I. Variations in stability of antigen-antibody complexes in hemophilia A. Blood 42: 365.
• 2 Didisheim P, Loeb J, Blatrix C, Soulier J. P. 1959; Preparation of a human plasma fraction rich in prothrombin, proconvertin, Stuart factor and PTC and a study of its activity and toxicity in rabbit and man. Journal of Laboratory and Clinical Medicine 53: 322.
• 3 Hruby M. A, Schulman I. 1973; Failure of combined factor VIII and cyclophosphamide to suppress antibody to Factor VIII in hemophilia. Blood 42: 919.
• 4 Meunier L, Allain J. P, Frommel D. 1975; Performance of an artificial reagent for the one-stage factor IX assay. Thrombosis et Diathesis Haemorrhagica 23: 547.
• 5 Nilsson I. M, Hedner U, Bjorlin G. 1973; Suppression of factor IX antibody in hemophilia B by factor IX and cyclophosphamide. Annals of Internal Medicine 78: 91.
• 6 Nilsson I. M, Hedner U, Holmberg L. 1974; Suppression of factor VIII antibody by combined factor VIII and cyclophosphamide. Acta Medica Scandinavica 195: 65.
• 7 Penner J. A, Kelly P. E. 1975; Management of patients with factor VIII or IX inhibitors. Seminars on Thrombosis and Hemostasis 1: 386.
• 8 Ruggeri Z. M, Mannucci P. M, Allain J. P, Frommel D. 1975; Preliminary trial of cyclophosphamide in the management of hemophiliacs with factor VIII inhibitors. Annals of the New York Academy of Sciences 240: 412.
• 9 Santos G. W. 1974; Immunosuppression for clinical bone marrow transplantation. Seminars in Hematology 11: 341.
• 10 Shapiro S. S, Hultin M. 1975; Acquired inhibitors to the blood coagulation factors. Seminars in Thrombosis and Hemostasis 1: 336.