Hemophilia Musculoskeletal Complications eMedicine Radiology

Description: Hemophilia is the oldest known bleeding disorder. This condition first came to public attention when the disease appeared in the offspring of Queen Victoria of England.
The deficiency or absence of either of 2 clotting elements — factor VIII or factor IX — leads to the clinical condition described as hemophilia A or hemophilia B, respectively. Hemophilia B was formerly known as Christmas disease and is not described in this article; in this condition, life-threatening bleeding into the head or the abdomen may occur at any age.
People with one of these bleeding disorders are prone to have recurrent episodes of hemorrhage into the joints. Acute bleeding increases the pressure in the synovial cavity and bone marrow, which leads to severe pain and possibly osteonecrosis or a pseudotumoral mass. Intra-articular bleeding produces a direct chemical effect on the synovium, cartilage, and bone. Over time, the blood becomes deposited in the form of hemosiderin in these tissues. Recurrent hyperemia of the joint in the growing child causes juxta-articular osteoporosis and overgrowth of the epiphysis.
Ray F Kilcoyne, MD Updated: Mar 21, 2008