RESUMEN
Acquired hemophilia A (AHA) is a rare hemorrhagic coagulopathy caused by the presence of autoantibodies that inhibit the activity of factor VIII (FVIII). Its diagnosis requires a high index of suspicion. It should be suspected in the presence of extensive hematomas or intense mucosal bleeding in patients with no history of previous trauma or hemorrhagic symptoms. We present two clinical cases of AHA, with different presentations and therapeutic management based on immunosuppression and hemostatic control through bypass agents such as activated recombinant FVII (rFVIIa; Novoseven®) and activated prothrombin complex concentrate (aPCC; Feiba®). The first case was an idiopathic AHA that presented with extensive subcutaneous hematomas with inhibitor titer >40 Bethesda units/ml (BU/mL), prolonged activated partial thromboplastin time (aPTT), and FVIII of 0.8%. In contrast, the second case involved a patient with a history of autoimmune disease, who presented with epistaxis and inhibitor titer of 10.8 BU/ml and FVIII of 5.3%.
RESUMEN
OBJECTIVE: To evaluate the efficacy and safety of a plasma-derived factor VIII concentrate containing von Willebrand Factor (pdVWF/FVIII) in standard clinical practice in von Willebrand Disease (VWD) patients. METHODS: A retrospective, multicentric, observational study of VWD patients treated with Fanhdi®, a pdVWF/FVIII concentrate, from January 2011 to December 2017 was conducted at 14 centers in Spain. Efficacy and safety were evaluated for acute bleeding episodes, for prevention of bleeding in surgeries, and for secondary long-term prophylaxis. RESULTS: Seventy-two eligible patients, type 1, 2, 3 VWD (25%/38.9%/36.1%) were treated for spontaneous and traumatic bleeding (140 episodes, n = 41 patients), to prevent surgical bleeding (69 episodes, n = 43 patients); and for secondary long-term prophylaxis (18 programs, n = 13 patients). Replacement therapy with pdVWF/FVIII showed an excellent to good clinical efficacy in 96.7% of the bleeding episodes, 100% during surgical procedures and 100% during prophylaxis. No adverse events (AEs), nor serious AEs related to the product were observed. CONCLUSIONS: Fanhdi® was effective, safe and well tolerated in the management of bleeding episodes, the prevention of bleeding during surgeries, and for secondary long-term prophylaxis in VWD patients.