OBSOLETE: Acquired hemophilia

Acquired hemophilia (also known as acquired hemophilia A when involving factor VIII) is a rare autoimmune bleeding disorder in which the body produces autoantibodies (called inhibitors) directed against one of its own clotting factors, most commonly factor VIII. Unlike inherited hemophilia, acquired hemophilia is not caused by a genetic mutation passed from parent to child but instead arises spontaneously, typically in older adults. It can be associated with underlying conditions such as autoimmune diseases, malignancies, pregnancy, or certain medications, though in approximately half of cases no underlying cause is identified (idiopathic). The condition affects the coagulation system, leading to potentially severe and life-threatening bleeding episodes. Key clinical features include extensive subcutaneous bruising (ecchymoses), muscle bleeding (hematomas), mucosal bleeding, gastrointestinal or urogenital hemorrhage, and prolonged bleeding after surgery or trauma. Unlike inherited hemophilia, joint bleeding (hemarthrosis) is relatively uncommon. The bleeding pattern tends to be diffuse and can be severe. Treatment involves two main strategies: controlling acute bleeding episodes and eradicating the inhibitor. Acute bleeding is managed with bypassing agents such as recombinant activated factor VII (rFVIIa) or activated prothrombin complex concentrates (aPCC). Inhibitor eradication is achieved through immunosuppressive therapy, typically corticosteroids alone or in combination with cyclophosphamide, rituximab, or other immunosuppressive agents. Early diagnosis and prompt treatment are critical, as mortality rates can be significant if the condition is not recognized and managed appropriately. Note: This Orphanet entry (ORPHA:73274) is marked as obsolete, and the condition may be catalogued under updated identifiers in current databases.

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