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Abstract
Objective: This review describes hemophila A and B, discusses the pathogenesis and genetic mechanisms associated with the disease, describes clinical and laboratory manifestations observed in patients, and lists treatment options for managing patients with hemophilia. Background: Hemophilia is a rare, congenital bleeding disorder with a X-linked recessive inheritance pattern. It is characterized by absent, decreased, or dysfunctional coagulation factor FVIII or FIX. Individuals with severe hemophilia bleed into the joints, soft tissue and muscles which can be debilitating. Hemophilia is classified as mild, moderate, and severe, and the degree of bleeding is proportional to the level of factor activity present. Conclusion: Even though hemophilia is a serious life-long bleeding disorder, understanding the pathogenesis of the disease leads to better patient management and improved patient outcomes.
- Received October 18, 2018.
- Revision received February 22, 2019.
- Accepted March 13, 2019.
- Published by American Society for Clinical Laboratory Science