RSS-Feed abonnieren
DOI: 10.1055/s-0038-1661062
The Importance of Corticoids Added to Continued Treatment with Factor VIII Concentrates in the Suppression of Inhibitors in Haemophilia A
Publikationsverlauf
Received 31. Oktober 1983
Accepted 01. Februar 1984
Publikationsdatum:
19. Juli 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00035024/198402/lookinside/thumbnails/10-1055-s-0038-1661062-1.jpg)
Summary
A protocol is presented to suppress inhibitors in haemophilia A using continued treatment with f. VIII (50 U/kg b.w./day) and fluprednisolone (0.5 mg/kg b.w., for 21 days) until reaching correct in vivo recovery after therapeutic administration.
5 high responder patients were treated whose inhibitors were detected at least 2 years before beginning the treatment. The inhibitors were eradicated in 4 patients using continued treatment for between 4 and 24 months. Patient 1 was treated exclusively with factor and the others also with corticoids when the level was 0 B.U. (or 20 B.U., patient 4). The patients began tri-weekly prophylactic treatment and rehabilitation exercises after recovery test normalization. After progressive reduction of the prophylactic treatment they started on demand treatment. After more than 5 months of this treatment no anamnestic response has been detected in any patient, in spite of several factor VIII administrations.
-
References
- 1 Shapiro SS, Hultin M. Acquired inhibitors to the blood coagulation factors. Semin Thromb Haemostas 1975; 1: 336-385
- 2 Biggs R. Jaundice and antibodies against factor VIII and IX in patients treated for Haemophilia or Christmas disease in the United Kingdom. Br J Haematol 1974; 26: 313-329
- 3 Brackmann HH, Etzel F, Hoffmann P, Egli H. The successful treatment of acquired inhibitors against factor VIII. Thromb Haemostas 1977; 38: 369 (Abstr.)
- 4 Brackmann HH, Gromsen J. Massive factor VIII infusion in a haemophiliac with factor VIII inhibitor, high responder. The Lancet 1977; 2: 933
- 5 Aznar JA, Jimenez C, Maicas M, Martin G, Marty ML. Estandarización de la técnica para la dosificación del factor VIII (en un tiempo). Estudio de su reproductibilidad Sangre 1980; 25: 1115-1118
- 6 Kasper CK, Aledort LM, Counts RB, Edson JR, Fratantoni J, Green D, Hampton JW, Hilgartner MW, Lazerson J, Levine PH, Mc Millan CW, Pool JG, Shapiro SS, Shulman NR, Van Eys J. A more uniform measurement of factor VIII inhibitors. Thrombos Diathes Haemorrh 1975; 34: 869-872
- 7 Biggs R, Bidwell E. A method for the study of antihaemophilic globulin inhibitors with reference to six cases. Br J Haematol 1959; 5: 379-395
- 8 Rizza CR, Biggs R. The treatment of patients who have factor VIII antibodies. Br J Haematol 1973; 24: 65-82
- 9 Ruggeri ZM, Zimmermann TS. The complex multimeric composition of factor VIII/von Willebrand factor. Blood 1981; 57: 1140-1143
- 10 Counts RB, Paskerll SL, Elgee SK. Disulfide bonds and the quaternary structure of factor VIII/von Willebrand factor. J Clin Invest 1978; 62: 702-707
- 11 Seghatchian MJ, Nilsson IM, Holmberg L, Miller-Anderson M. Molecular size distribution of factor VIII in native plasma. Thromb Res 1979; 14: 589-598
- 12 Lavergne JM, Meyer D, Girma JP, Larrieu MJ. Precipitating antifactor VIII:C antibodies in two patients with Haemophilia A. Br J Haematol 1982; 50: 135-146
- 13 Holmberg L, Borge L, Nilsson IM. F VIII:C and VIIIC:Ag response in patients with haemophilia A and von Willebrand disease after administration of different factor VIII concentrates and plasma. Br J Haematol 1981; 47: 587-598
- 14 Blatt PM, White GC, McMillan CW, Roberts HR. Treatment of anti-factor VIII antibodies. Thromb Haemostas 1977; 38: 514-523
- 15 Allain J, Frommel DAntibodies. Patterns of immune response to factor VIII in hemophilia A. Blood 1976; 47: 973-982
- 16 Pintado T, Taswell HF, Bowie EJ. Treatment of life threatening hemorrhage due to acquired factor VIII inhibitor. Blood 1975; 46: 535-541
- 17 Slocombe GW, Newland AC, Colvin MP, Colvin BT. The role of intensive plasma exchange in the prevention and management of haemorrhage in patients with inhibitors to factor VIII. Br J Haematol 1981; 47: 577-585
- 18 Haemophilia study group. Efficacy of Prothrombin complex concentrates in haemophiliacs with antibodies to factor VIII. A multicenter therapeutic trial N Engl J Med 1981; 303: 421-425
- 19 Barrowclife TW, Kemball-Cook G, Gary E. Factor VIII inhibitor bypassing activity. A suggested mechanism of action Thromb Res 1981; 21: 181-186
- 20 Stenbjerg S, Jorgensen J. Disseminated intravascular coagulation and infusion of factor VIII inhibitor bypassing activity. Lancet 1977; 1: 360
- 21 Stenbjerg S, Tauris P, Skottun T, Jorgensen J. Total dental extraction in a patient with F VIII inhibitor. Thromb Res 1980; 18: 889-893
- 22 Kasper CK, Feinstein DI. Rising factor VIII inhibitor titers after Konyne factor IX complex. N Engl J Med 1976; 295: 505-506
- 23 Palaskak JE, Shapiro SS. Antifactor VIII anamnesis after factor IX complex. N Engl J Med 1977; 297: 1403
- 24 Green D, Lechner K. A Survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemostas 1981; 45: 200-203
- 25 Dormandy KM, Sultan Y. The suppression of factor VIII antibodies in haemophilia. Pathol Biol 1975; 23: 17-23
- 26 Hultin MB, Shapiro SS, Bowman HS, Gill FM, Andrews AT, Martinez J, Eyster ME, Sherwood WC. Immunosuppressive therapy of factor VIII inhibitors. Blood 1976; 48: 95-108
- 27 Stein RS, Colman RW. Hemophilia with factor VIII inhibitors: Elimination of anamnestic response. Ann Intern Med 1973; 79: 84-87
- 28 Hruby MA, Schulman I. Failure of combined factor VIII and cyclophosphamide to suppress antibody to factor VIII in hemophilia. Blood 1973; 42: 919-923
- 29 Aznar JA, Jorquera JI, Peiro A. Suppression of inhibitors in hemophilia with corticoids and factor VIII. Thromb Haemostas 1983; 49: 248
- 30 Bloom AL. Clotting factor VIII concentrates for resistant haemophilia. Br J Haematol 1978; 40: 21-27