Hemophilia A in Females: Considerations for Clinical Management.


Journal

Acta haematologica
ISSN: 1421-9662
Titre abrégé: Acta Haematol
Pays: Switzerland
ID NLM: 0141053

Informations de publication

Date de publication:
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

289-294

Informations de copyright

© 2019 S. Karger AG, Basel.

Auteurs

Paulette Bryant (P)

St Jude Affiliate Clinic at Novant Health Hemby Children's Hospital, Charlotte, North Carolina, USA.

Aikaterini Boukouvala (A)

Arizona Bleeding Disorders Health and Wellness Center, Phoenix, Arizona, USA.

Jenny McDaniel (J)

St Jude Affiliate Clinic at Novant Health Hemby Children's Hospital, Charlotte, North Carolina, USA.

Danielle Nance (D)

Banner MD Anderson Cancer Center, Gilbert, Arizona, USA, danielle.nance@bannerhealth.com.

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Classifications MeSH