Prophylaxis in Patients with Severe Hemophilia and Inhibitor
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Prophylaxis in Patients with Severe Hemophilia and Inhibitor
Victor Jimenez‐Yuste 1 2, Maria Teresa Alvarez 2, Monica Martin‐Salces 2, E Carlos Rodriguez‐Merchan 3, Nora Butta 4, Ihosvany Fernandez‐Bello 4, Isabel Rivas 4 and Ana Rodriguez de la Rua 2
Affiliations: 1Department of Hematology, Autonoma University, Madrid, Spain; 2Hematology Department, HospitalUniversitario La Paz, Madrid, Spain; 3Department of Orthopedics and Hemophilia Unit, Autonoma University, Madrid, Spain; 4Research into Hemostasis Unit, Hospital Universitario La Paz, Madrid, Spain
Submission date: 4th July 2009, Revision date: 4th August 2009, Acceptance date: 17th September 2009
ABSTRACT
Joint hemorrhage, the most common manifestation of severe hemophilia, frequently leads to recurrent hemarthrosis, chronic synovitis, and arthropathy. The main goal of replacement therapy is to prevent this pathology. On‐demand treatment slows, but does not prevent, the progression of arthropathy. It has been shown that prophylaxis is superior to enhanced, episode‐based therapy in preventing joint damage. However, patients often develop antibodies that inhibit or neutralize factor VIII (FVIII) or factor IX (FIX) replacement therapy in what is today the most serious complication of hemophilia and its treatment. The effects of bypassing agents in treating the bleeding episodes are not as predictable as those of replacement therapy with the deficient factor in patients with inhibitors. Consequently, these patients have higher levels of arthropathy than patients without inhibitors. Prophylaxis for patients with inhibitors has gained attention over the last decade, and some papers have reported that bypassing agents may help to prevent arthropathy. Nevertheless, the justification of prophylaxis in patients with inhibitors is still questioned because of concerns regarding its cost, complications, and efficacy.
Keywords: Haemophlia, inhibitors, prophylaxis, arthropaty
Correspondence: Victor Jimenez‐Yuste, Servicio de Hematología y Hemoterapia, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046 Madrid, Spain
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