Factor VIII inhibitors in hemophilia A: rationale and latest evidence-Reference-Cited by-同舟云学术

Factor VIII inhibitors in hemophilia A: rationale and latest evidence

Published:2012-10-30 Issue:1 Volume:4 Page:59-72
ISSN:2040-6207
Container-title:Therapeutic Advances in Hematology
language:en
Short-container-title:Therapeutic Advances in Hematology

Author:

Witmer Char¹,Young Guy²

Affiliation:

1. Division of Hematology, The Children’s Hospital of Philadelphia, 3501 Civic Center Boulevard, CTRB 11th Floor, Room 11026, Philadelphia, PA 19104, USA

2. Division of Hematology/Oncology, Children’s Hospital Los Angeles and University of Southern California Keck School of Medicine, Los Angeles, CA, USA

Abstract

Factor VIII (FVIII) replacement therapy is the foundation of treatment in hemophilia A and is effective unless a patient develops an alloantibody (inhibitor) against exogenous FVIII. Inhibitor development is currently the most significant treatment complication seen in patients with hemophilia and is associated with considerable morbidity and a decreased quality of life. The development of an inhibitor is the result of a complex interaction between a patient’s immune system and genetic and environmental risk factors. The mainstay of treatment is the eradication of the inhibitor through immune tolerance. This review summarizes the current evidence regarding inhibitor risk factors, eradication, and hemostatic bypassing agents.

Publisher

SAGE Publications

Subject

Hematology

Link

http://journals.sagepub.com/doi/pdf/10.1177/2040620712464509

Reference119 articles.

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3. Why do inhibitors develop? Principles of and factors influencing the risk for inhibitor development in haemophilia

4. The Malmö International Brother Study (MIBS): further support for genetic predisposition to inhibitor development

5. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FEIBA NovoSeven Comparative (FENOC) Study

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