Hemophilia encompasses a group of hereditary bleeding disorders characterized by coagulation dysfunction, which predominantly affects males. Caused by a deficiency in coagulation factor VIII (FVIII) or factor IX (FIX), this condition impairs the generation of active thromboplastin and prolongs coagulation time. Consequently, patients exhibit a bleeding tendency following minor trauma; in severe cases, “spontaneous” bleeding can occur even in the absence of obvious injury. Hemophilia is classified into mild, moderate, or severe based on plasma factor levels of 6% to 40%, 1% to 5%, and less than 1%, respectively. In patients with severe hemophilia, whose FVIII or FIX activity is less than 1% of normal levels, frequent joint bleeding episodes (hemarthrosis) may occur 20 to 30 times annually.
Lifelong factor replacement therapy is crucial for prolonging the life expectancy of patients with hemophilia and reducing musculoskeletal bleeding. However, complications such as musculoskeletal bleeding may still arise due to insufficient factor dosage, poor medication adherence, or the development of neutralizing antibodies (inhibitors) targeted against FVIII or FIX. The ankle, elbow, and knee joints are the most common sites for hemarthrosis in these patients. The inflammatory response triggered by recurrent intra-articular bleeding leads to acute and chronic pain, cartilage damage, joint degeneration, and functional loss, eventually progressing to severe osteoarthritis. It is currently reported that 90% of hemophilia patients will develop hemophilic arthropathy within 30 years of onset. For chronic hemophilic synovitis, chemical and radionuclide synovectomy can delay the progression to hemophilic arthropathy, whereas severe hemophilic arthropathy warrants surgical intervention. Total knee arthroplasty (TKA) is an highly effective procedure for alleviating pain and restoring function in patients with advanced hemophilic arthropathy of the knee. This article reviews the latest literature regarding the perioperative management, intraoperative considerations, prosthesis selection, postoperative rehabilitation, and associated complications of TKA in patients with hemophilia.
