Clinical Scenarios in Hospitalized Patients With Hemophilia

住院血友病患者的临床情景

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Abstract

Hemophilia is an X-linked recessive bleeding disorder caused by a deficiency of coagulation factor VIII (hemophilia A) or factor IX (hemophilia B). Despite therapeutic advances that have extended life expectancy, hospitalized patients with hemophilia present complex clinical challenges. This narrative review, illustrated with four structured clinical cases, addresses the most common scenarios encountered in the management of hospitalized patients with hemophilia: acute bleeding, perioperative management without inhibitors, perioperative management with inhibitors, and antithrombotic therapy in patients with comorbid cardiovascular disease. Factor replacement remains the cornerstone of therapy, with target levels and dosing duration determined by bleeding site and procedure type. Inhibitor development occurs in up to 30% of hemophilia A patients and necessitates the use of bypassing agents (recombinant activated factor VII or activated prothrombin complex concentrate). Perioperative management requires a multidisciplinary approach with 24-hour laboratory and blood bank availability. Cardiovascular comorbidities, including ischemic heart disease and atrial fibrillation, occur at rates comparable to the general population in aging patients with hemophilia; antithrombotic therapy must be individualized according to baseline factor levels, bleeding phenotype, and inhibitor status. Left atrial appendage closure represents a viable alternative to long-term anticoagulation in selected high-risk patients. Optimal management of hospitalized patients with hemophilia demands individualized, multidisciplinary care. Clinicians should be familiar with factor replacement protocols, inhibitor detection and management strategies, and the growing challenge of balancing bleeding risk with antithrombotic therapy in an aging hemophilia population. These practical considerations are essential for hospital-based clinicians involved in the care of this complex patient group.

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