Hemophilia A in Females: Considerations for Clinical Management
- 1 June 2020
- journal article
- research article
- Published by S. Karger AG in Acta Haematologica
- Vol. 143 (3), 289-294
- https://doi.org/10.1159/000503889
Abstract
Approximately 50% of female carriers of hemophilia A have factor VIII (FVIII) levels below 0.5 IU/dL and may be categorized as having mild hemophilia. Females with hemophilia may go undiagnosed for years because the most common symptoms - menorrhagia and bleeding after childbirth - also occur in females without hemophilia. Females with hemophilia can exhibit increased bleeding tendencies despite current guidelines of expected, adequate FVIII levels. The cases described illustrate the clinical variability and presentation of hemophilia in females and highlight the importance of a timely diagnosis, effective management, and monitoring. Prophylactic factor replacement therapy is recommended in females with hemophilia, particularly those with joint disease or gynecologic complications. Affected individuals should receive infusion training and education on treatment options, physical activities, the importance of treatment adherence, and recognizing bleeding symptoms warranting treatment. Further study is needed to increase awareness of hemophilia in females and reassess current guidelines for their management and monitoring.Keywords
This publication has 14 references indexed in Scilit:
- Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and BBlood Coagulation & Fibrinolysis, 2014
- Both Hemophilia Health Care Providers and Hemophilia A Carriers Report That Carriers Have Excessive BleedingJournal of Pediatric Hematology/Oncology, 2014
- Severe and moderate haemophilia A and B in US femalesHaemophilia, 2014
- Correlation between phenotype and genotype in a large unselected cohort of children with severe hemophilia ABlood, 2013
- The experience of girls and young Women with inherited bleeding disordersHaemophilia, 2013
- Guidelines for the management of hemophiliaHaemophilia, 2012
- Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment CentresHaemophilia, 2011
- Association between phenotype and genotype in carriers of haemophilia AHaemophilia, 2010
- Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe HemophiliaNew England Journal of Medicine, 2007
- Bleeding in carriers of hemophiliaBlood, 2006