Haemophilic arthropathy occurs due to recurrent bleeding into joints leading to swelling, inflammation, destruction of cartilage and bone, and development of arthritis. Although prophylactic replacement therapy assists in preventing arthropathy, it is not always adequate or affordable. Radiosynovectomy is a minimally invasive intervention for treatment of synovitis in haemophilic joints. The procedure utilises locally injected radioisotopes (Y, P, Rh) to ablate abnormal synovium with the goal of decreasing bleeding, slowing progression of cartilage and bone damage and preventing arthropathy. The objective of this review is to summarize the radiosynovectomy literature and to present patient outcomes associated with radiosynovectomy over the past 17 years from two haemophilia treatment centers (HTCs), one in the United States and one in Spain. Articles from these two centers support the current literature. A retrospective medical records review was performed by the two reporting HTCs on patients who underwent radiosynovectomy prior to 2009. Data review included: site of procedure, isotope utilized, bleeding frequency, and procedure complications. Radiosynovectomy is a cost-effective, minimally invasive, well tolerated procedure. As the paradigm for care in haemophilia shifts towards prevention of joint disease, the number of target joints with synovitis will likely decrease, except in patients who develop inhibitors. We propose early consideration of radiosynovectomy for patients with haemophilic synovitis prior to appearance of articular cartilage damage.
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http://dx.doi.org/10.1097/MBC.0b013e3283602a28 | DOI Listing |
Cureus
December 2024
Department of Medicine, Assam Medical College and Hospital, Dibrugarh, IND.
Background and objective Hemophilia A (HA) is a genetic bleeding disorder caused by a lack of factor VIII (FVIII) and is associated with frequent bleeding and joint damage. Traditional intravenous treatments for this condition are cumbersome and can lead to complications. Emicizumab, a bispecific monoclonal antibody, offers a promising subcutaneous alternative with potential safety and efficacy-related benefits.
View Article and Find Full Text PDFZhongguo Shi Yan Xue Ye Xue Za Zhi
December 2024
Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology, Shijiazhuang 050000, Hebei Province, China.
Objective: To explore the diagnosis and treatment of acquired hemophilia A (AHA) based on the analysis of clinical data.
Methods: A retrospective analysis was conducted on the clinical manifestations, laboratory characteristics, treatment, and outcomes of 25 patients diagnosed with AHA who were admitted to the Second Hospital of Hebei Medical University.
Results: Among all patients, 11 cases had secondary factors, including 5 cases of autoimmune diseases, 3 cases of pregnancy-related disease, 1 case of pemphigoid, 1 case of Graves' disease, and 1 case of monoclonal gammaglobulinemia of unknown significance (MGUS).
J Thromb Haemost
December 2024
Department of Clinical Sciences Lund, Lund University, Lund, Sweden; Center for Thrombosis and Hemostasis, Skåne University Hospital, Malmö, Sweden. Electronic address:
Background: A unique form of Hemophilia B (HB) is HB Leyden. We evaluated the International PedNet Registry database to explore the natural history of HB Leyden, investigate genotype-phenotype associations and guide clinical decision-making.
Objectives: To assess the association between genetic variants, endogenous factor (FIX) levels over time, treatment and bleeding phenotype in children with HB Leyden.
Haemophilia
December 2024
Advanced Center for Oncology, Hematology and Rare Disorders (ACOHRD), K.J. Somaiya Super Speciality Hospital & Research Center, Somaiya Ayurvihar, Sion East, Mumbai, Maharashtra, India.
Introduction: Mortality and morbidity in persons with haemophilia (PWH) have decreased due to improved diagnosis and treatment along with comprehensive population outreach efforts, but the impact is not uniform in different countries.
Aim: The study aims to assess all-cause and intracranial haemorrhage (ICH)-specific mortality of PWH in India.
Methods: This is a retrospective, observational, multi-centric cohort study of 1020 haemophilia patients from three centres in India.
Rev Bras Ortop (Sao Paulo)
November 2024
Departamento de Cirurgia Ortopédica, Fundación Cardioinfantil, Instituto de Cardiología, Bogotá, Colômbia.
Patients with hemophilia disease have a high risk of hemorrhage. Most hemorrhages can occur in the musculoskeletal system, presenting as hematomas, or, in rare occasions, as hemophilic pseudotumors, an uncommon pathology that are often misdiagnosed as musculoskeletal tumors because of their clinical behavior and characteristics on diagnostic imaging. Despite many treatment options, surgical excision is the treatment of choice.
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