Life-threatening bleeding in a case of autoantibody-induced factor VII deficiency.
ABSTRACT A male patient presented with life-threatening bleeding induced by autoantibody-induced factor VII (F.VII) deficiency. This patient had macroscopic hematuria, skin ecchymosis, gastrointestinal bleeding, and a neck hematoma that was causing disturbed respiration. He developed acute renal failure and acute hepatic failure, probably due to obstruction of the ureters and the biliary tract, respectively. Although activated partial thromboplastin time was normal, prothrombin time (PT) was remarkably prolonged at 71.8 seconds compared to 14.0 seconds in a normal control. Both the immunoreactive level of F.VII antigen and the F.VII activity of the patient's plasma samples were < 1.0% of normal. Although an equal part of normal plasma was added to the patient's plasma, PT was not corrected. The patient's plasma inhibited F.VII activity. These findings suggested the presence of a plasma inhibitor for F.VII. After administration of large doses of methylprednisolone, PT was gradually shortened and plasma levels of F.VII increased over time. Bleeding, acute renal failure, and acute hepatic failure improved markedly following the steroid treatment. These observations suggest that life-threatening bleeding can be induced by autoantibody-induced F.VII deficiency and that immunosuppressive therapy using large doses of steroid can be successful in inhibiting the production of the autoantibody.
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ABSTRACT: Acquired factor VII (FVII) deficiency in the absence of vitamin K deficiency, oral anticoagulant therapy, synthetic liver dysfunction, or DIC is rare, with only a handful of cases thus far reported. In the period from 1990 to 1996 we identified eight patients with acquired FVII deficiency, all of whom presented with prolongation of the prothrombin time (PT) in the first 2 weeks following stem cell transplantation (SCT). The mean plasma FVII clotting activity (FVII:c) was 22% (range 8-35%) with an approximately equivalent reduction in FVII antigen (FVII:Ag) level. Mean plasma levels of fibrinogen and factors II, V, IX, and X were normal. Protein C activity was significantly depressed in only one of the three patients in whom it was measured. Several patients experienced bleeding complications, and hemorrhage directly accounted for death in two cases. Veno-occlusive disease of the liver developed in three patients. We conclude that FVII deficiency should be considered in the differential diagnosis of prolonged PT in patients who have recently undergone SCT. The mechanism of this acquired deficiency state remains to be defined.Bone Marrow Transplantation 04/2002; 29(5):403-8. DOI:10.1038/sj.bmt.1703381 · 3.47 Impact Factor
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ABSTRACT: Acquired isolated FVII deficiency not due to vitamin K deficiency or liver disease is rare and often associated with severe bleeding. We present a case of transient acquired factor VII deficiency associated with major bleeding, successfully treated with twice daily intermittent intravenous recombinant activated factor VII (rFVIIa) (NovoSeven; Novo Nordisk). The severe transient reduction in factor VII coagulant activity (FVII:C) levels, unresponsive to fresh frozen plasma and vitamin K administration, raise the possibility of an acquired inhibitor to factor VII. However, no inhibitor to factor VII could be demonstrated using protein G sepharose adsorption, or a Bethesda assay using IgG purified from patient plasma. There are few reports of the use of rFVIIa in this setting and this case suggests that rFVIIa is effective therapy, and should be considered early when acquired factor VII deficiency is associated with severe bleeding.Blood Coagulation and Fibrinolysis 07/2004; 15(4):347-51. DOI:10.1097/00001721-200406000-00010 · 1.38 Impact Factor
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ABSTRACT: We report a case of transient acquired and isolated factor VII deficiency associated with severe head trauma. A 16-year-old boy was involved in a motor vehicle accident. CT scan showed frontal brain contusion and a cerebral haematoma (5 cm). First prothrombine time (PT) was normal. Rapidly, a severe coagulopathy developed, unresponsiving to fresh frozen plasma and vitamin K. Haemostatic markers analysis showed an isolated deficiency of factor VII at 15%. No inhibitory activity against factor VII could be detected. We successfully treated the deficiency with intermittent intravenous human factor VII (factor VII-LFB®) during 10 days. Factor VII return to normal at 84%. Physiopathological and therapeutic aspects of this rare pathology are presented.Annales Françaises d Anesthésie et de Réanimation 11/2005; 24(11):1383-1386. DOI:10.1016/j.annfar.2005.05.019 · 0.84 Impact Factor