PB2323: ACQUIRED HEMOPHILIA: A CASE REPORT

Background: Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies (inhibitors) against endogenous factor VIII (FVIII) that can lead to life-threatening bleeding. It may be secondary to an underlying disease or idiopathic. The annual incidence is 1.5 cases per million per year, with two peaks in frequency: in postpartum women (2 to 15%) and in patients aged over 60 (75%). 80% of patients have extensive unexplained bruising requiring urgent laboratory exploration. Mortality due to misfortune is between 8 and 22%. Aims: we discuss a case of acquired hemophilia A, presenting as spontaneous bruising in an elderly patient. Methods: This is an 80-year-old man, with a history of type I diabetes and hypertension, referred to our laboratory for exploration of extensive bruising, which appeared spontaneously. The questioning did not reveal any personal or family history of abnormal bleeding, or the use of any anticoagulant or antiplatelet medication. Results: Hemostatic tests of the patient revealed isolated prolonged aPTT to 59 sec (control 29 sec) with a normal PT. The measurement of the FVIII activity revealed a level of 4.5 % (normal value 60–150%). Accordingly, factor VIII inhibitors were detected at 04 Bethesda units (BU). Recombined FVIIa have been used in the treatment of bleeding episode. Immunosuppressive treatment with rituximab was administered in order to remove the factor VIII inhibitor. After 04 cures of rituximab, the aPTT wase normalised (patient 30 sec/control 30 sec) which means a total removal of factor VIII inhibitor. Summary/Conclusion: Diagnosis of Acquired hemophilia A is considered a challenge given its nonspecific presentation and low prevalence worldwide.