Table 79–1 shows a clinical classification of hemophilia A based on factor VIII levels.
Hemostasis is generally normal with levels in excess of 30 percent.
The factor VIII level remains constant throughout the patient's life, and is similar in other affected members of the kindred, but varies between kindreds.
Hemarthrosis accounts for 75 percent of bleeding episodes in patients with severe hemophilia A.
The most frequent sites are the knees, followed by the elbows, ankles, shoulders, wrists, and hips.
The acute form is characterized by initial mild pain without physical findings, followed by more intense pain, swelling and warmth of the joint, and decreased range of motion.
The patient may have mild fever. Significant or sustained fever suggests infection in the joint.
When bleeding stops, the blood resorbs and symptoms subside over several days.
Repeated bleeding into the joint results in synovial hypertrophy and inflammation, with limitation of motion and a tendency for more frequent bleeding in that joint (target joint).
Eventually, repeated hemorrhage into the joints causes destruction of the articular cartilage, synovial hyperplasia, and joint deformity with muscle atrophy and soft tissue contractures (Fig. 79–2).
Hematomas may develop after bleeding into muscles or subcutaneous ...