How I use bypassing therapy for prophylaxis in patients with hemophilia A and inhibitors

Author:

Leissinger Cindy A.1,Singleton Tammuella1,Kruse-Jarres Rebecca2

Affiliation:

1. Louisiana Center for Bleeding and Clotting Disorders, Tulane University Medical Center, New Orleans, LA; and

2. Washington Center for Bleeding Disorders, Bloodworks Northwest, Seattle, WA

Abstract

Abstract Inhibitor development poses a significant challenge in the management of hemophilia because once an inhibitor is present, bleeding episodes can no longer be treated with standard clotting factor replacement therapy. Consequently, patients with inhibitors are at increased risk for difficult-to-control bleeding and complications, particularly arthropathy and physical disability. Three clinical trials in patients with inhibitors have demonstrated that prophylaxis with a bypassing agent reduces joint and other types of bleeding and improves health-related quality of life compared with on-demand bypassing therapy. In hemophilia patients without inhibitors, the initiation of prophylaxis with factor (F) VIII or FIX prior to the onset of recurrent hemarthroses can prevent the development of joint disease. Whether this is also true for bypassing agent prophylaxis remains to be determined.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference75 articles.

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2. Kasper CK Diagnosis and management of inhibitors to factors VIII and IX. An introductory discussion for physicians. Treatment of Hemophilia Monograph Series, no. 34; September 2004. Available at: http://www1.wfh.org/publication/files/pdf-1178.pdf. Accessed August 29, 2014

3. Treatment-related risk factors of inhibitor development in previously untreated patients with hemophilia A: the CANAL cohort study.;Gouw;Blood,2007

4. Factor IX inhibitors and anaphylaxis in hemophilia B.;Warrier;J Pediatr Hematol Oncol,1997

5. Diagnosis and quantification of factor VIII inhibitors.;Verbruggen;Haemophilia,2010

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