Hemophilic Arthropathy: Barriers to Early Diagnosis and Management

Abstract

Hemophilia is a congenital coagulopathy characterized by a deficiency of one of the clotting factors. It is characterized by the development of hematomas and hemarthrosis, either spontaneously or after minor trauma. The recurrence of hemarthroses leads to progressive and degenerative joint damage from childhood (hemophilic arthropathy). This arthropathy is characterized by disabling physical effects that limit the functionality and quality of life of these patients. Medical progress achieved over the last decade in the drug treatment of hemophilia has improved the medium and long-term prospects of patients with more effective and long-lasting drugs. The universal use of safer, more effective and prolonged prophylactic treatments may promote the prevention of bleeding, and also therefore, of the development of hemarthrosis and joint damage. A number of imaging instruments have been developed for the assessment of hemarthrosis and hemophilic arthropathy, using ultrasound, magnetic resonance imaging and simple radiology. Different physical examination scores and questionnaires allow the assessment of joint health, self-perceived activity and functionality of patients with hemophilia. The approach to these patients should be interdisciplinary. Assessment of the processes that affect pain in these patients and the development of pain education models should be implemented. Expert advice and information to patients with hemophilia should be based on individual functional prevention diagnoses, advice on available therapies and sports practice, as well as health recommendations.

Haemophilic arthropathy: Diagnosis, management, and aging patient considerations

“…Surgical synovectomy, open or arthroscopic, is indicated for patients with subacute or chronic synovitis with no response to medical treatment for at least 6 months and with moderate radiological scores. 49,50,59 Relative contraindications include high neutralising inhibitor titer and advanced arthropathy. Although > 80% effective in decreasing subsequent haemarthrosis, open synovectomy is expensive, requires a prolonged hospital stay and haemostatic factor replacement, and comes with several possible serious complications including loss of movement, infection, stiffness, fracture and postoperative haemarthrosis.…”

Stromal cell‐derived factor 1 alpha (SDF‐1alfa) and cartilage oligomeric matrix protein (COMP): Two potential signature biomarkers of radiological detectable hemophilic arthropathy

“…Patients with joint damage exhibit a spectrum of radiographic changes, starting with mild subchondral irregularity and cyst formation, advancing to joint space narrowing, osteophyte formation, marginal erosion, and eventual deformity. 6 In order to improve an early diagnosis of hemophilic arthropathy and appreciate articular lesions, more performing tools are needed, that would be easy to use, of reasonable cost, and good sensitivity, to detect articular lesions in association with imaging methods.…”

“…However, is less sensitive than MRI and its interpretation is more operator‐dependent. Patients with joint damage exhibit a spectrum of radiographic changes, starting with mild subchondral irregularity and cyst formation, advancing to joint space narrowing, osteophyte formation, marginal erosion, and eventual deformity 6 …”

A Retrospective, Long‐term Follow‐up of the Clinical Outcomes and Cost‐effectiveness of Single‐anesthetic Multiple Total Joint Procedures in Hemophilic Arthropathy

“…The condition is debilitating and recurrent haemarthrosis causes repetitive synovial inflammation and, consequently destruction of the joint tissues 2 . HA can affect almost every joint, primarily the weight‐bearing joints such as knees, ankles, and hips 3,4 …”

“…2 HA can affect almost every joint, primarily the weight-bearing joints such as knees, ankles, and hips. 3,4 Different surgical management approaches have been reported in the literature, including ankle debridement, synovectomy, arthrodesis, and total joint replacement (TJR), which is considered the most significant procedure. 5 TJR, including total knee arthroplasty (TKA) and total hip arthroplasty (THA), is now frequently applied in severe cases as an optimal procedure to alleviate pain and restore joint functions.…”