Case Report: Acquired Hemophilia by the presence of anti-factor VII autoantibodies associated with an antiphospholipid syndrome: a case report and review of literature

Abstract

Introduction Acquired hemophilia is a rare disease characterized by the presence of neutralizing autoantibodies against hemostasis factors, most often factor VII. However, a few cases of anti-factor VII (FVII) autoantibodies have been reported in the literature. We report a case in this regard. Case report A 28-year-old woman with no family or personal history presented with a severe hemorrhagic syndrome made of very abundant metrorrhagia with multiple compressive cervical hematomas, associated with pulmonary embolism and thrombosis. of the right external iliac vein. On the biological assessment, the patient presented with an undetectable PT with a factor VII titer less than 6%. The hemorrhagic syndrome was aggravated by infusions of activated factor VII (FVIIa) with the appearance of hemoptysis and hematomas in all four limbs. Etiological assessment concluded the presence of anti-factor VII autoantibodies as well as circulating lupus anticoagulant. Other antiphospholipid syndrome (APS) antibodies as well as antibodies against other hemostasis factors were absent. A treatment combining corticosteroid therapy (1mg per kg per day of prednisone) and Mycophenolate mofetil (MMF) (3g) was initiated. Faced with the persistence of the hemorrhagic syndrome, rituximab was administered according to the rheumatological protocol (1g on D1 and 1g on D15). Metrorrhagia improved initially but recurred with the resumption of menstruation with a drop in hemoglobin from 9 to 7 g/dl. Plasmapheresis sessions were attempted but thromboses on the catheters occurred each time. Immunoglobulin (IVIg) courses were administered. No anticoagulant was administered. TP increased to 23%. Levels of neutralizing antibodies fell from 512 to 2 IBU at the last follow-up at five months from the bleeding event. The circulating lupus anticoagulant was present on the follow-up test after 12 weeks, thus confirming the diagnosis of APS. Conclusion Our patient presents an extremely rare case of acquired haemophilia. The combination of corticosteroid therapy, conventional and biological immunosupressives, IVIG and plasmapheresis saved the patient. In the absence of consensus on the treatment, it remains adapted according to the severity of the haemorrhagic syndrome and the associated comorbidities.