[Recombinant activated Factor VII (Novoseven Novo Nordisk) for hemostasis in acquired Factor VIII-inhibitor hemophilia].
One life threatening mediastinal hemorrhage and two limb threatening hemorrhages, one in the retroperitoneal and thigh muscles and the other in the back of the hand requiring surgical evacuation, were treated with recombinant activated factor VII concentrate (rFVIIa; Novoseven Novó Nordisk) in a patient with a postpartum acquired inhibitor against factor VIII. High dose activated prothrombin complex concentrate (Feiba sTIM4 Immuno), repeatedly administered, had proven to be ineffective; porcine factor VIII concentrate (Hyate C Porton) had become ineffective due to a rise in inhibitor titers against human and porcine factor VIII as well. 90 micrograms rFVIIa per kg body weight was administered as an i.v. bolus injection every 2-3.5 hours. The treatment periods were 22.5 days in the mediastinal and 11 days in each of the two other hemorrhages. Hemostasis was promptly achieved and maintained. All manipulations (bone marrow biopsy, surgical evacuation of the hematoma, change of venous access, withdrawal of drains, change of dressings) were done immediately after rFVIIa administration, without bleeding complications. There were no side effects despite the high dose, frequent and long lasting treatment with a total of 234 x 4.8 and 46 x 3.6 mg rFVIIa. The concentrate was well tolerated, there were no signs of systemic activation of coagulation, either in the coagulation test results or clinically, in spite of patient's factor VII levels up to 60 U/mL and prothrombin times around 6 s. No inhibitors against patient's factor VII, induced by rFVIIa, were detected. Due to its extrinsic factor VIII bypass effect, rFVIIa led to excellent hemostasis even with inhibitor titers of 376 Bethesda units against human and 44 against porcine factor VIII. Nevertheless, immunosuppressive treatment with cyclophosphamide and prednisone was performed, with prompt decrease of the inhibitor titer.(ABSTRACT TRUNCATED AT 250 WORDS)
Meili
E O
EO
Departement für Innere Medizin, Universitätsspital Zürich.
Dazzi
H
H
von Felten
A
A
D000328:Adult; D005169:Factor VIII / Q000037:antagonists & inhibitors; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000628:therapy*; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D011247:Pregnancy; D011644:Puerperal Disorders / Q000628:therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use
D011994:Recombinant Proteins; D005169:Factor VIII; D015942:Factor VIIa
Rekombinanter aktivierter Faktor VII (Novoseven Novo Nordisk) zur Blutstillung bei erworbener Hemmkörper-Hämophilie.