Hemophilia A in Females: Considerations for Clinical Management.
Adolescent
Aged
Chromosome Inversion
Deamino Arginine Vasopressin
/ adverse effects
Disease Management
Drug Substitution
Factor VIII
/ analysis
Female
Hemarthrosis
/ etiology
Hemophilia A
/ complications
Heterozygote
Humans
Introns
/ genetics
Male
Menorrhagia
/ etiology
Middle Aged
Pedigree
Recombinant Proteins
/ therapeutic use
Tranexamic Acid
/ therapeutic use
Young Adult
Females
Hemophilia A
Management
Journal
Acta haematologica
ISSN: 1421-9662
Titre abrégé: Acta Haematol
Pays: Switzerland
ID NLM: 0141053
Informations de publication
Date de publication:
2020
2020
Historique:
received:
07
06
2019
accepted:
04
10
2019
pubmed:
12
12
2019
medline:
25
8
2020
entrez:
12
12
2019
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 31825922
pii: 000503889
doi: 10.1159/000503889
doi:
Substances chimiques
Recombinant Proteins
0
Tranexamic Acid
6T84R30KC1
F8 protein, human
839MOZ74GK
Factor VIII
9001-27-8
Deamino Arginine Vasopressin
ENR1LLB0FP
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
289-294Informations de copyright
© 2019 S. Karger AG, Basel.