Objective: The aims of this study were (1) to document the prevalence of acute hemarthrosis in a cohort of 46 boys with severe hemophilia A receiving full primary prophylaxis in Western Australia (WA), and (2) to investigate the safety of the WA protocol over 11 years for management of hemarthrosis.
Methods: Case review. The WA protocol involves a pediatric rheumatologist washing out all acute hemarthrosis of large joints promptly and then instilling intraarticular (IA) corticosteroids.
Results: This study showed that joint bleeds occurred in 22 boys of 46 (47.8%). In over 11 years, 84 washouts were performed on 32 joints in 22 boys. No adverse events occurred. Fifteen of 22 boys had normal joints with a Hemophilic Joint Health Score = 0. Fifteen boys who had had all hemarthrosis washed out had clinically normal joints (100%). Seven boys had sustained joint damage prior to full instigation of the protocol, each having had documented hemarthrosis without aspiration. Parents needed to understand that joint bleeds constituted an emergency.
Conclusion: Of our cohort, 47.8% of patients with severe hemophilia receiving prophylaxis developed joint bleeding. The WA protocol is safe. There is evidence suggesting joint outcomes of hemophilic patients having hemarthrosis despite factor VIII prophylaxis may be much improved if there is access to a center using a procedure similar to the WA protocol.
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http://dx.doi.org/10.3899/jrheum.141236 | DOI Listing |
Haemophilia
December 2024
Department of Medicine, University of Colorado School of Medicine, Aurora, Colorado, USA.
Introduction: People with haemophilia A (PwHA) experience acute and chronic pain associated with reduced quality of life (QoL).
Aims: This post hoc analysis of pooled data from the HAVEN 1 (NCT02622321), 3 (NCT02847637), 4 (NCT03020160) and STASEY (NCT0319179) studies assessed the impact of emicizumab prophylaxis on pain-related QoL in PwHA.
Methods: PwHA received emicizumab during the four studies.
Appl Sci (Basel)
July 2024
Department of Biomedical Engineering, Columbia University, New York, NY 10027, USA.
Recurrent bleeding in the synovial joint, such as the knee, can give rise to chronic synovitis and degenerative arthritis, which are major causes of morbidity. Whereas chronic arthropathy affects one-fifth of hemophiliacs, conditions such as rheumatoid arthritis (RA), periarticular and articular fractures, osteochondral autograft transplantation surgery, and anterior cruciate ligament (ACL) injury are also associated with joint bleeding. Synovial joint trauma is associated with inflammation, acute pain, bloody joint effusion, and knee instability.
View Article and Find Full Text PDFHealthcare (Basel)
October 2024
Orthopedics and Traumatology Clinic, IRCCS Policlinico San Matteo Foundation, 27100 Pavia, Italy.
Background: Hemophilia type A and B is associated with spontaneous bleeding in muscle tissues and joints. Acute hemarthrosis, representing 70-80% of all bleedings in severe hemophilia patients, is extremely painful. When surgical procedures are needed in hemophiliac patients, perioperative management should be planned with a multidisciplinary team.
View Article and Find Full Text PDFHaemophilia
November 2024
University of North Carolina School of Medicine, Capel Hill, North Carolina, USA.
Introduction: The management of bleeding events (BEs) in haemophilia A (HA) and B (HB) patients with inhibitors necessitates the use of bypassing agents. The recombinant factor VIIa bypassing agent eptacog beta has demonstrated efficacy at treating BEs and managing perioperative bleeding in adults in phase three clinical studies.
Aim: To provide real-world descriptions of eptacog beta use for BE treatment in patients on emicizumab or eptacog beta prophylaxis.
Expert Rev Hematol
August 2024
Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago, IL, USA.
Introduction: Hemophilia is an inherited bleeding disorder. Bleeding, and in particular joint hemorrhage results in chronic arthropathy and disability. Acute and chronic pain are frequent and limit activity and participation and result in decreased health-related quality of life.
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