Severe acquired haemophilia A treated with recombinant factor VIIa

Abstract

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...

Modulation of the Coagulation Cascade Using Recombinant Factor VIIa and Activated Protein C in a Severely Injured Trauma Patient

“…Since its introduction into clinical practice in 1988, the majority of experience with rFVIIa has been in patients with hemophilia A or B, hemophiliacs who have developed factor inhibitors, factor VII deficiency, thrombocytopenia, and platelet disorders [15][16][17][18]. More recently, the use of rFVIIa has expanded to include control of hemorrhage related to abdominal surgery, orthopedics, urology, liver transplant, and cardiac surgery [18][19][20][21][22][23][24][25].…”

Hemorrhaging during pregnancy

“…Mehrere Fallberichte zeigten, dass die Applikation von rFVIIa eine suffiziente thera peutische Option ist, diese Akutproblematik zu beherrschen [24,58].…”

Intestinal surgery with activated recombinant factor VII prophylaxis in patients with haemophilia A and high responding inhibitors

“…Few published data are available on rFVIIa prophylaxis during surgery on the central nervous system [10,12] and other types of surgery, such as urologic [8,14], ophthalmologic [10,15], abdominal [16] and intestinal surgery [9,10,14,[17][18][19]. Here we describe the use of rFVIIa prophylaxis during intestinal surgery in five patients with haemophilia A and high responding inhibitors.…”