Acquired Hemophilia

Excerpt

Hemophilia is derived from hemo (blood) and philia (love), is recognized as the most common and severe hemorrhagic disorder. Depending upon the pathophysiology, there are three types of hemophilia -A, B, and C, caused by the deficiency or dysfunction of factors VIII, IX, and XI, respectively. It can be hereditary, which is more common, and a rare variety is the acquired one. Acquired hemophilia is due to the autoantibodies that develop against a coagulation factor. Mostly the antibodies develop against the factor VIII, hence commonly known as "acquired hemophilia A" (AHA).

Patients with autoantibodies may present with catastrophic bleeding; they usually do not have any past medical history of bleeding episodes or disorder. Its diagnosis is often difficult because of its rarity and the complexity of the laboratory workup. This article describes the clinical and biological features, in addition to the therapeutic strategies for this disease.

Clinically, the presentation varies from life-threatening bleeds on one end to mild or no bleeds on the other end. The presentation with life-threatening bleeding is usually seen during the initial several weeks, although it can happen at any point during disease if the appropriate treatment is not initiated.