Acquired haemophilia A : case report and literature review.
Hemofilia A adquirida : reporte de un caso y revisión de la literatura.
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Acquired haemophilia A (AHA) is a very rare autoimmune disease characterized by presence of autoantibodies to circulating factor VIII; it manifests clinically as a hemorrhagic syndrome, especially with mucocutaneous, subcutaneous and soft tissues bleeding. Etiology is idiopathic in most cases; the other possible etiologies include collagen diseases, solid tumors, postpartum, hematolymphoid malignancies and drugs. Mortality depends on age, being majority of deaths in elderly people. We described a 93-years-old women presenting with a severe subcutaneous bleeding showing a prolonged partial thromboplastin time. In the diagnostic approach we found a diminished factor VIII-activity and high inhibitor titer (120 Bethesda units/ml) without a previous history of coagulation disorders. We initiated treatment with corticosteroids and then we added cyclophosphamide to achieve a reduction in the inhibitor titers and short the aPTT, however, patient died after three weeks due to an ischemic stroke unrelated to the prescribed treatment.
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