Erworbene Faktor Vlll·C-Hemmkörper bei Nichthämophilen
| Autor: | M. Heins, B. M. E. Kuntz, Dieter Bach, Wolfgang Schneider, M. Burk, Ch. Specker, Carlo Aul, D. Söhngen |
|---|---|
| Rok vydání: | 1995 |
| Předmět: | |
| Zdroj: | Transfusion Medicine and Hemotherapy. 22:56-59 |
| ISSN: | 1660-3818 1660-3796 |
| Popis: | Antibodies against factor VIII:C occur in about 5-15 % of hemophilia A patients and induce refractoriness to factor VIII:C infusions. These antibodies are mostly of IgG class. In very rare cases factor VIII:C inhibitors also develop in nonhemophilic patients. Although there are anecdotal reports that these antibodies may disappear spontaneously, in the majority of patients the clinical course is characterized by severe hemorrhages. During the last ten years we treated 9 nonhemophilic patients with acquired factor VIII:C inhibitors at our clinic. In most cases, a sudden bleeding tendency was observed shortly after an injury or surgery. Coagulation tests showed a prolonged aPTT and a decreased F VIIL:C level. Therapy with F VIII·C concentrates did not produce the expected increase. Measurement of F VIII:C inhibitor levels (Bethesda Units = BU) revealed values in the range between 2 and 108 BU. Immunosuppressive therapy in patients with > 5 BU using azathioprine or cyclo-phosphamide in combination with prednisone lead to complete disappearance of the inhibitor, normalization of the coagulation tests, and complete remission of the bleeding tendency in 7 patients within 6 weeks. During pregnancy only prednisone should be given. On the other hand in women with post-partum factor VIII inhibitors, steroid treatment appears to be not superior to no treatment, but immunosup-pression with cyclophosphamide or azathioprine seems to be more effective. In emergency situations, therapy with high doses of human F VIII:C concentrate is recommended. When bleeding does not cease, the additional use of activated pro-thrombin concentrates or porcine factor VIII concentrate is indicated. Side effects may include hepatitis and short-lived intravascular thrombin production. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|