Joint problems caused by chronic bleeding often trouble people with hemophilia, especially in the knees, ankles, and elbows. A paper by scientists in Spain and the U.S. reviewed existing literature on knee treatments for people with this disease, focusing on best current practices — including a preventive, if inconveniently lifelong, therapy — and future treatment directions.
The article, “Orthopedic disorders of the knee in hemophilia: A current concept review,” was published in the World Journal of Orthopedics.
Hemophilia’s two forms are caused by deficiencies in clotting proteins, factor VIII (FVIII) for hemophilia A and factor IX (FIX) for hemophilia B. According to a recent study reported in Hemophilia News Today, these problems may be more severe in people with hemophilia A than those with hemophilia B.
The study’s lead authors, E. Carlos Rodriguez-Merchan of the Department of Orthopedic Surgery, La Paz University Hospital-IdiPaz, Madrid, and Leonard A. Valentino of Rush University Medical Center, Chicago, report that factor replacement therapy (FRT) is the optimal approach for preventing knee and other joint problems (known as hemophilic arthropathy), through infusions to replace the deficient protein. But use of FRT therapy is lifelong, making adherence a challenge, and breakthrough bleeding is common. Surgery can correct damage to the knee, but is difficult in people with this bleeding disorder.
If FRT therapy is insufficient, hemophilic synovitis can develop, a condition affecting the synovial membrane that lines joints such as the knee. Rodriguez-Merchan and Valentino recommend radiosynovectomy as the best way to reduce bleeding in people with this condition. This involves injections of radioactive nucleotides into the knee. The method can replace surgical synovectomy (the removal of the synovial membrane).
The researchers recommend that these knee surgeries be performed in specialized hospitals, with experienced teams of healthcare providers, to minimize the heightened risk of bleeding and infection.
“Until we are able to prevent orthopedic problems of the knee” in people with hemophilia, we “will have to continue performing orthopedic procedures (arthrocentesis, RS [radiosynovectomy], arthroscopic synovectomy, hamstring release, arthroscopic debridement, alignment osteotomy, and total knee arthroplasty)” to improve patients’ quality of life, the authors wrote.
But, they conclude: “The best treatment … is primary prophylaxis [FRT therapy] replacing the deficient clotting factor with early institution of regular injections of concentrates of factor VIII or IX. In this way, not only is bleeding into the joints prevented but also the development of synovitis and articular degeneration.”