Abstract
Hemophilia A is a hereditary, X-linked recessive bleeding disorder caused by deficiency or dysfunction of coagulation factor Ⅷ (FⅧ) in plasma. Patients with severe hemophilia A (SHA) have FⅧ activity<1% and are prone to frequent spontaneous bleeding particularly in joints and muscles. Recurrent joint bleeding can lead to chronic arthropathy, and then affect activities of daily living and quality of life of these patients. Prophylaxis, namely regular infusion of coagulation factor concentrate, has been demonstrated effectiveness in preventing hemarthrosis in children with SHA and is recommended as the management of choice by the World Federation of Hemophilia (WFH). However, its role in adults with SHA is still debated. In this review, authors focus on the benefits, cost, individualization and evaluation tools of the prophylaxis in adult patients with SHA. Key words: Severe hemophilia A; Adult; Tertiary prevention
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