Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by autoantibodies against FVIII. Severe AHA is life-threatening. Currently, licensed hemostatic agents for the treatment of severe AHA have short half-lives and require intravenous administration, leading to a need for hospitalization, higher costs, and negative effects on quality of life. We present two cases of severe AHA with high inhibitor titers where emicizumab was safely and effectively used with intensive immunosuppression. These reports suggest in vivo efficacy even in high inhibitor environments. The optimal dosing regimen (accelerated vs. standard loading, maintenance frequency) is unknown and we discuss the current approaches.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11020103 | PMC |
| http://dx.doi.org/10.1002/jha2.878 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Dental management of people with congenital hemophilia: An integrative review of case reports and case series from a global scenario.
Spec Care Dentist
December 2024
Department of Child Health Nursing, Manipal College of Nursing, Manipal Academy of Higher Education, Manipal, Karnataka, India.
Objective: To analyze the various dental management strategies adopted to manage patients with hemophilia in a dental clinical setup.
Methods: An electronic database search was carried out using MEDLINE by PubMed, Scopus, Google Scholar, Web of Science, and EMBASE databases from January 2000 to August 2023 for case reports and case series published in English language. Case reports addressing the dental treatments for people with hemophilia A/hemophilia B were included.
Pharmacokinetic Studies, Assessing the Efficiency of FVIII/VWF Concentrates and Intravenous Human Immunoglobulin, Revealed the Etiopathogenesis of Acquired von Willebrand Disease in Patient With MGUS.
Haemophilia
December 2024
Department of Molecular Medicine and Medical Biotechnology, University of Naples "Federico II", Naples, Italy.
Real-World Data on Patients With Acquired Haemophilia A in Japan Undergoing Rehabilitation or With Low Activities of Daily Living Scores: The ORIHIME II Study.
Haemophilia
December 2024
Department of Pediatrics, Nara Medical University, Kashihara, Japan.
Introduction: Acquired haemophilia A (AHA) is characterized by the development of autoantibodies against factor VIII, reducing its activity and potentially resulting in bleeding.
Aim: To assess the characteristics of people with AHA undergoing rehabilitation and/or with low activities of daily living (ADL) scores, thereby characterizing unmet needs in the management of AHA and informing treatment optimization.
Methods: ORIHIME II, the largest epidemiological and treatment survey of AHA in Japan, is a descriptive, retrospective, observational study conducted using health claims data from April 2008 to October 2021.
Platton S, Sivapalaratnam S, Raheja P. Diagnosis and laboratory monitoring of acquired hemophilia A. Hematology Am Soc Hematol Educ Program. 2023;2023(1):11-18.
Hematology Am Soc Hematol Educ Program
December 2024
Safety assessment of tafamidis: a real-world adverse event analysis from the FAERS database.
Expert Opin Drug Saf
December 2024
Department of Pharmacy, Pingtan Comprehensive Experimental Area Hospital, Fuzhou, China.
Background: Tafamidis emerged as the first FDA-approved drug for treating termed amyloid fibrils. This study aims to analyze adverse events (AEs) related to tafamidis from the second quarter (Q2) of 2019 to the fourth quarter (Q4) of 2023 from the FDA adverse event reporting system (FAERS) database.
Research Design And Methods: The AE data related to tafamidis from 2019 Q2 to 2023 Q4 were collected and standardized.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!