Hemophilia A yang Didapat: Suatu Tinjauan tentang Diagnosis dan Manajemen

Abstract

Acquired hemophilia A (AHA) is a rare acquired bleeding disorder, an autoimmune disease characterized by autoantibody directed against factor VIII. Typically, patients with no prior history ofbleeding disorder, present with spontaneous bleeding, especially skin and mucosal bleeding. AHA occurs more frequently in the elderly, both in male and female and may be associated with several conditions, such as autoimmune diseases (systemic lupus erythematosus, lymphoproliferative disease, rheumatoid arthritis), malignancies and pregnancy. Around half of the patients are idiopathic. AHA is diagnosed in patients without previous personal or family bleeding history in which prolonged activated partial thromboplastin time is not corrected after mixing and incubating for 2 – 4 hours at 37oC equal volumes of patient and normal plasma. FVIII:C levels are reduced and a specific FVIII inhibiting activity is detected and measured by the Bethesda assay or its Nijmegen modification. In the management of AHA, control of acute bleeding is the first priority. In patients with low level inhibitor, desmopressin may be used. But FVIII by passing agents (activated prothrombin complex concentrate and recombinant activated FVII) are required for patients with high titre of antibody or with serious bleeding episode. Immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. It is recommended initial treatment with corticosteroid or combination therapy with corticosteroid and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy is fails or is contraindicated.

Keywords

• acquired hemophilia a
• diagnosis
• management