Acquired Hemophilia

Acquired hemophilia A is a disorder of rare entity, resulting in spontaneous bleeding in individuals with no history of bleeding disorders. It is believed to be caused by spontaneous inhibition of clotting factor VIII by autoantibodies and is usually associated with other autoimmune conditions.

The hallmark of this condition is mucocutaneous bleeding leading to ecchymosis, melena, hematoma or hematuria. The bleeding associated with AHA tends to be in mucocutaneous sites or soft tissues and can lead to recurrent gastrointestinal, intramuscular, or intracranial bleeding in elderly patients as compared to hemarthrosis in younger patients with congenital hemophilia.

Hemophilia A

Hemophilia A is a rare X-linked congenital bleeding disorder characterized by a deficiency of coagulation factor VIII (FVIII).

Hemophilia A is estimated to account for 80%-85% of all hemophilia cases.

Hemorrhages, musculoskeletal complications, and other sequelae of hemophilia typically occur in males with hemophilia but may also occur in a proportion of female carriers.

Hemophilia should be suspected in individuals presenting with a history of any of these symptoms: easy bruising; “spontaneous” bleeding (i.e., bleeding for no apparent/known reason), particularly into the joints, muscles, soft tissues; and/or excessive bleeding following trauma or surgery.

Hemophilia A or B with Inhibitors

“Inhibitors” in hemophilia are IgG alloantibodies to exogenous clotting factor VIII (FVIII) or factor IX (FIX) that neutralize the function of infused clotting factor concentrates (CFCs).

Inhibitors are more frequently encountered in patients with severe disease than in those with moderate or mild hemophilia, and more commonly in patients with hemophilia A than in those with hemophilia B. Controlling bleeds is a greater challenge in hemophilia patients with inhibitors than in those without inhibitors.

Inhibitors to FVIII or FIX are associated with a higher disease burden, including increased risk of musculoskeletal complications, pain, physical limitations, and treatment challenges, all of which may impact a patient's physical functioning, capacity for physical activities, and quality of life.

Hemophilia B

Hemophilia B is a rare X-linked congenital bleeding disorder characterized by a deficiency of coagulation factor IX (FIX).

Hemophilia B is estimated to account for 15%-20% of all hemophilia cases.

Hemorrhages, musculoskeletal complications, and other sequelae of hemophilia typically occur in males with hemophilia but may also occur in a proportion of female carriers.

Hemophilia should be suspected in individuals presenting with a history of any of these symptoms: easy bruising; “spontaneous” bleeding (i.e., bleeding for no apparent/known reason), particularly into the joints, muscles, soft tissues; and/or excessive bleeding following trauma or surgery.