Thromb Haemost 1981; 45(03): 200-203
DOI: 10.1055/s-0038-1650169
Original Article
Schattauer GmbH Stuttgart

A Survey of 215 Non-Hemophilic Patients with Inhibitors to Factor VIII

David Green
The Atherosclerosis Program, Department of Medicine, Northwestern University-Rehabilitation Institute of Chicago, U. S. A. and the I. Medizinische Universitätsklinik, Vienna, Austria
,
Klaus Lechner
The Atherosclerosis Program, Department of Medicine, Northwestern University-Rehabilitation Institute of Chicago, U. S. A. and the I. Medizinische Universitätsklinik, Vienna, Austria
› Institutsangaben
Weitere Informationen

Publikationsverlauf

Received 23. Oktober 1980

Accepted 23. Januar 1981

Publikationsdatum:
26. Juli 2018 (online)

Summary

Information was obtained by questionnaire about 215 nonhemophilic patients who developed inhibitors against factor VIII (antihemophilic factor). The majority of the patients were over 50 years of age, and approximately equal numbers of males and females were reported. Rheumatoid arthritis was present in 8% of the cases, 7% occurred during pregnancy or the post-partum period, and in several there was an association with allergy to penicillin, asthma, “auto-immune” diseases, or malignancy. In 46% of cases, no underlying disorders were identified. Major bleeding was observed in 87 % of patients, and in 22%, death was attributed either directly or indirectly to the presence of the inhibitor.

In 11 of 31 patients receiving no therapy other than supportive transfusions of blood or factor VIII concentrate, the inhibitor disappeared after being present for an average duration of 14 months. Corticosteriods were thought to be effective in abolishing the inhibitor in 22 of 45 patients in whom these were the only drugs administered. Twenty-eight patients received azathioprine as well as corticosteriods; in 19, the inhibitor declined or disappeared during treatment. Finally, 80 patients were treated with cyclophosphamide; in 37 there was a favorable outcome. Inhibitors in children and post-partum patients were more likely to disappear spontaneously or with steroid therapy, whereas those in patients with rheumatoid arthritis or other “autoimmune” disorders required treatment with alkylating agents. However, before any specific therapy can be recommended for this disorder, prospective trials of potential therapeutic agents should be conducted in selected subgroups.

 
  • References

  • 1 Kasper CK. A more uniform measurement of factor VIII inhibitors. Thromb Diath Haemorrh 1975; 34: 869-872
  • 2 Austen DE G, Rhymes IL. A Laboratory Manual of Blood Coagulation. Blackwell Scientific Publications; Oxford: 1975. p 68-69
  • 3 Green D. Spontaneous inhibitors of factor VIII. Brit J Haemat 1968; 15: 57-75
  • 4 Spero JA, Lewis JH, Hasiba U. Corticosteroid therapy for acquired factor VIII:C inhibitors. Blood 1979; 54: 304a (Abs.)
  • 5 Lechner K. Acquired inhibitors in non-hemophilic patients. Haemost 1974; 03: 65-93
  • 6 Green D, Schuette PT, Wallace WH. Factor VIII antibodies in rheumatoid arthritis: effect of cyclophosphamide. Arch Int Med 1980; 140: 1232-1235
  • 7 Rizza CR, Edgcumbe JOP, Pitney WR, Child JA. The treatment of patients having spontaneously occurring antibodies to antihemophilic factor (factor VIII). Thromb Diath Haemorrh 1972; 28: 120-128
  • 8 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