Objective: Activated prothrombin complex concentrate (aPCC) is a bypassing agent indicated to treat bleeds in patients with acquired hemophilia A (AHA). Nevertheless, its efficacy and safety in the real-world setting have not often been addressed.
Methods: We report the experience of Spanish reference centers for coagulation disorders and from acquired hemophilia Spanish Registry (AHASR) from August 2012 to February 2021. Follow-up period of 30 days after aPCC withdrawal.
Results: Thirty patients with a median age of 70 years old, suffering from 51 bleeds treated with aPCC were finally evaluated. As first-line treatment, aPCC stopped bleeding in 13 of 14 (92.9%) cases. aPCC as the second line after recombinant factor VIIa failure, stopped bleeding in all cases. In 17 patients, aPCC was used far from initial bleed control as prophylaxis of rebleeding with 94% effectiveness. No thromboembolic episodes were communicated. One patient developed hypofibrinogenemia, which did not prevent aPCC from halting bleeding. No other serious adverse events possibly or probably associated with aPCC were reported.
Conclusions: This data support aPCC as hemostatic treatment in AHA with high effectiveness and excellent safety profile in acute bleeds and as extended use to prevent rebleedings, even in aging people with high cardiovascular risk.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1111/ejh.13853 | DOI Listing |
Hemophilia A is a clotting disorder due to factor VIII deficiency, leading to prolonged bleeding. Acquired hemophilia A results from the immune system attacking factor VIII, typically occurring later in life. Factor V Leiden is a genetic mutation causing abnormal blood clot formation, primarily in veins.
View Article and Find Full Text PDFHaemophilia
January 2025
Katharine Dormandy Haemophilia and Thrombosis Unit, Royal Free Hospital, London, UK.
Background: Acquired haemophilia A (AHA) is an acquired bleeding disorder resulting from autoantibodies against Factor VIII (FVIII). Previous studies have reported differences in FVIII inhibitor kinetics (type I or type II) in AHA compared to severe haemophilia A.
Aim: To characterise inhibitor kinetics in AHA and evaluate the proportions displaying type I, II or indeterminate kinetics.
Res Pract Thromb Haemost
January 2025
Department of Pediatric Hematology, Innovative Hematology, Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana, USA.
Background: The development of acquired factor (F)V with inhibitor (AFVwI) is rare, resulting mainly in bleeding complications, although sporadic cases of thrombosis in adults have been reported.
Key Clinical Question: How do you diagnose and manage a pediatric case of acute deep venous thrombosis associated with the concurrent finding of AFVwI?
Clinical Approach: A 13-year-old female with Crohn's Disease and May-Thurner anatomy developed extensive deep venous thrombosis of the left lower extremity, complicated by the finding of AFVwI, discovered during the evaluation of a prolonged prothrombin time and a low FV activity. Anticoagulation was initiated with low-molecular-weight heparin followed by a direct oral anticoagulant, rivaroxaban, without any complications.
J Clin Med
December 2024
Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW 2145, Australia.
This perspective discusses the critical role of laboratory assessments in assessing factor VIII (FVIII) inhibitors. These are auto- and alloantibodies that can develop against both endogenous and exogenous FVIII, respectively. Assessment for inhibitors represents a key part of the management of both congenital hemophilia A (CHA), an inherited deficiency, and acquired hemophilia A (AHA), an autoimmune condition.
View Article and Find Full Text PDFTransplant Proc
January 2025
Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. Electronic address:
Background: Liver transplantation is an important treatment option for liver cirrhosis in patients with HIV/HCV coinfection. In Japan, the limited number of deceased donors may force the selection of living donor liver transplantation. Appropriate graft selection is the key to success.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!