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April 30, 1973

Section 48.—Hemophila

JAMA. 1973;224(Suppl_5):771. doi:10.1001/jama.1973.03220190111051

Hemarthrosis occurs in 80% to 90% of patients with hemophilia and constitutes the most common major hemorrhagic event in this disorder. Joint bleeding usually begins before the age of 5 and tends to recur repeatedly during childhood, after which it becomes less frequent. Hemarthrosis usually results from injury although the trauma may be trivial. The knees, elbows, and ankles (including subtalar joints) are the parts most often affected. Shoulders, hips, wrists, fingers, and toes are less frequently involved.1

In acute hemarthrosis the joint becomes painful, swollen, warm, and markedly tender, and there may be fever and leukocytosis. When bleeding is mild, joints return to normal in a few days, but with severe hemorrhage inflammation may persist for weeks or months. Repeated hemarthroses lead to hemosiderosis of the synovium (Fig 77), degeneration of articular cartilage, thickening of periarticular tissues, bony hypertrophy, and deformities, most commonly flexion contractures of elbows and