When and how to start prophylaxis in boys with severe hemophilia without inhibitors: communication from the SSC of the ISTH.

Rituximab for Treatment of Hemophilia A with High-Responder Inhibitors

Background: The development of inhibitors is a complication factor replacement therapy in hereditary factor VIII deficiency. Several management options are available for the treatment of inhibitor. Rituximab, a monoclonal antibody against CD20, reduces inhibitor level in rare bleeding disorders. The aim of this study was to evaluate the effectiveness of rituximab in lowering or eliminating the ...

Design of clinical trials for new products in hemophilia: communication from the SSC of the ISTH.

Detection of Factor VIII Inhibitors in Hemophilia A Patients

Background: Factor VIII administration to hemophilia A patients results in an immune response (inhibitor formation) which significantly complicates the therapy. The present study was performed to determine the prevalence of inhibitor development in hemophilia A patients receiving recombinant factor VIII therapy. Materials and Methods: This was an observational descriptive study. Clotting fac...

Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.

BACKGROUND Effective ways to prevent arthropathy in severe hemophilia are unknown. METHODS We randomly assigned young boys with severe hemophilia A to regular infusions of recombinant factor VIII (prophylaxis) or to an enhanced episodic infusion schedule of at least three doses totaling a minimum of 80 IU of factor VIII per kilogram of body weight at the time of a joint hemorrhage. The primar...

The Survey of Effective Agents on Factor VIII and IX Inhibitors in Patients with Hemophilia A and B in Kermanshah Province

  Background: Hemophilia is the most frequent severe hereditary hemorrhagic disease due to deficiency of coagulation factors VIII (Hemophilia A) or IX (Hemophilia B) in plasma. We aimed to identify patients with  hemophilia in Kermanshah, Iran and assess the incidence of inhibitors in this population and its associated factors. Methods: This study was conducted on patients with hemophilia...