The use of recombinant factor VIIa in a patient with acquired haemophilia A undergoing surgery

Doughty, Heidi; Northeast, A.; Sklair, L.; Roques, Tom; Young, A. E.; Savidge, G. F.; Hunt, B J

doi:10.1097/00001721-199504000-00006

Cited by 16 publications

(11 citation statements)

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“…Recombinant FVIIa has been used in the management of acquired haemophilia with varying degrees of success (Cohen & Kessler, 1996;Doughty et al, 1995;Lusher et al, 1996;Majundar et al, 1993;Stein & Rantoff, 1993). We found that it arrested bleeding quickly and without sideeffects.…”

Section: Discussionmentioning

confidence: 99%

Severe acquired haemophilia A treated with recombinant factor VIIa

et al. 1997

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Summary. Acquired haemophilia can be associated with various conditions including pregnancy, autoimmune diseases and lymphoproliferative disorders, though often no underlying cause is found. It often presents with a rapid onset of muscle bleeding and involves the IgG antibody. It may be treated with human or porcine factor VIII (FVIII), prothrombin complex concentrates, factor IX (FIX) complex concentrates, factor VIIa (FVIIa) or by immunosuppression. We report a case of acquired haemophilia in a 40-year-old woman diagnosed following laparotomy. She was treated unsuccessfully using human FVIII and cryoprecipitate, porcine FVIII and FIX complex concentrate, before being treated with recombinant FVIIa (NovoSeven, Novo Nordisk). On treatment with recombinant FVIIa, bleeding stopped rapidly with no side-effects and the abdominal haematoma was evacuated with minimal post-operative bleeding.Keywords: severe acquired haemophilia, recombinant factor VIIa.A previously healthy 40-year-old Egyptian woman, with no history of a bleeding disorder which included an uncomplicated caesarean section for the delivery of her first baby, was referred to us as a case of acquired haemophilia for further management. This diagnosis was established following a laparotomy for an ectopic pregnancy when she was noted to bleed excessively both during surgery and post-operatively. She had a high activated partial thromboplastin time (aPTT) of 133 s/36 s due to the presence of high titres of factor VIII:C (FVIII:C) inhibitor (110 Bethesda units/ml). She had been managed with relatively high doses of human FVIII and cryoprecipitate without success. She continued to ooze intraabdominally and at the abdominal wall wound site.On arrival, apart from bleeding from the laparotomy site, she had no other complaints. On examination, she was pale but haemodynamically stable. There was evidence of bleeding from the operation site despite pressure dressings. There was a large bruise in the hypogastrium and groin region with underlying tenderness. The rest of the physical examination was normal.Investigations confirmed a high aPTT (87 s/36 s) rising to 104 s on day 4, due to a high titre of FVIII:C inhibitor (112 Bethesda units/ml) with a FVIII:C level of < 1%. Inhibitory antibody against porcine FVIII could not be measured due to the unavailability of reagents. The prothrombin time (PT) was normal. White cell and platelet counts were normal, post transfusion haemoglobin (Hb) was 9 . 1 g/dl. Serial axial CT scans of the abdomen confirmed the presence of two intraabdominal haematomas (Fig 1), one was related to the right rectus sheath measuring 3 × 4 × 10 cm and the other was anterior to the mesentry and measured 5 × 6 × 15 cm. These two haematomas and an intramural haematoma of the distal ileal loops were the major contributory factors in the development of intestinal obstruction which was managed conservatively. TREATMENTSince factor VIIa (FVIIa) was not immediately available, treatment was commenced with intravenous tranexamic acid and hydrocortisone. Pac...

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Section: Discussionmentioning

confidence: 99%

Severe acquired haemophilia A treated with recombinant factor VIIa

et al. 1997

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“…Supraphysiologisch dosierter rFVIIa kann somit das teilweise oder vollständige Fehlen der FVIII-oder FIXWirkung kompensieren ("Bypass-Effekt"), wodurch sich die hohe Wirksamkeit von rFVIIa bei der Behandlung und Prophylaxe von Blutungsepisoden bei Patienten mit angeborener oder erworbener Hämophilie erklärt [27]. Da [14,51]. Die hereditäre und die erworbene HK-Hämophilie sind bis dato die einzigen Krankheitsbilder, für die die Therapie mit rFVIIa zugelassen ist.…”

Section: Wirkungsmechanismus Von Rfviiaunclassified

“…Eine simultane Verabreichung von rFVIIa und aktivierten Prothrombinkomplexkonzentraten sollte nur unter strenger Nutzen-Risiko-Abwägung vorgenommen werden, da sich in einem Kaninchenmodell gezeigt hat, dass die thrombogene Wirkung von aktiviertem Prothrombinkomplexkonzentrat durch gleichzeitige rFVIIa-Gabe potenziert wird [14].…”

Section: Sicherheit Nebenwirkungen Und Kontraindikationen Von Rfviiaunclassified

Rekombinanter Faktor VIIa (NovoSeven®) Ein Überblick über aktuelle und mögliche zukünftige Indikationen

Heuer¹,

Blumenberg²

2002

Anaesthesist

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Recombinant activated coagulation factor VII (rFVIIa, NovoSeven) was originally developed for the treatment of bleeding complications in haemophilia patients with allo-antibodies (inhibitors) against exogenous factor VIII or IX. In 1988, rFVIIa was used successfully in such patients for the first time. Subsequently, the efficacy and safety of rFVIIa in haemophilia patients with inhibitors has been proven in several prospective trials. A large number of case reports and results from initial clinical trials suggest that rFVIIa may also be effective in the prevention and treatment of bleeding in patients under oral anticoagulation, with liver diseases, and in patients without any pre-existing haemorrhagic diathesis. However, further clinical studies will be necessary to specify the future potential of rFVIIa.

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“…Current management strategies aim to arrest bleeding through the use of factor VIIIc concentrates (either high‐dose human FVIIIc or porcine FVIIIc, depending upon inhibitor titre) [1, 5]. Individuals with high‐inhibitor titres against both human and porcine FVIIIc may require treatment with bypassing agents or recombinant FVIIa [6, 7]. Immunomodulatory treatment is used to attempt suppression or elimination of the inhibitor.…”

Section: Introductionmentioning

confidence: 99%

Acute renal failure complicating high-dose intravenous immunoglobulin therapy for acquired haemophilia

et al. 1999

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Acquired haemophilia is a rare disorder requiring therapy to control bleeding and to suppress the inhibitory antibody. High-dose intravenous immunoglobulin is commonly used as part of immunosuppressive regimens for this condition. We describe the case of an elderly patient who developed acute oliguric renal failure as a result of intravenous immunoglobulin therapy. All patients receiving such treatment should have renal function carefully monitored both during and after the infusion.

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The use of recombinant factor VIIa in a patient with acquired haemophilia A undergoing surgery

Cited by 16 publications

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Severe acquired haemophilia A treated with recombinant factor VIIa

Severe acquired haemophilia A treated with recombinant factor VIIa

Rekombinanter Faktor VIIa (NovoSeven®) Ein Überblick über aktuelle und mögliche zukünftige Indikationen

Acute renal failure complicating high-dose intravenous immunoglobulin therapy for acquired haemophilia

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